Review Series: Aspects of Interstitial lung disease: Connective tissue disease-associated interstitial lung disease: How does it differ from IPF? How should the clinical approach differ?
Free accessReview articleFirst published online February, 2011
Review Series: Aspects of Interstitial lung disease: Connective tissue disease-associated interstitial lung disease: How does it differ from IPF? How should the clinical approach differ?
The lung is frequently involved in connective tissue diseases (CTDs), although the frequency of lung manifestations varies according to the type of CTD. Interstitial lung diseases (ILD) are frequently seen in CTDs, particularly systemic sclerosis (SSc), polymyositis/dermatomyositis (PM/DM) and rheumatoid arthritis (RA), accounting for a significant proportion of deaths. A large percentage of patients with CTD-associated ILD has limited and stable disease, not requiring treatment. However, a significant minority has severe and/or progressive disease, necessitating prompt initiation of treatment. CTD-ILD histological patterns include non-specific interstitial pneumonia (NSIP), usual interstitial pneumonia (UIP), organizing pneumonia (OP), diffuse alveolar damage (DAD) and lymphocytic interstitial pneumonia (LIP). NSIP is the most common pattern in all CTDs, except for RA, characterized by a higher frequency of UIP. ILD can present acutely or chronically, with acute presentations being more common in systemic lupus erythematosus and PM/DM. Idiopathic pulmonary fibrosis (IPF) is a progressively worsening ILD characterized by inflammation and fibrosis. The characteristic histological pattern of IPF is UIP. Interestingly, a UIP pattern is associated with a significantly better survival in CTD-related disease compared to the idiopathic variety. Prognosis in IPF is dismal, with a median survival since diagnosis of 2-3 years. No treatment regimen has been shown to improve survival in IPF. By contrast, although there have been only two randomized placebo-controlled trials investigating the effect of immunosuppressive treatment in SSc-associated ILD, clinical experience suggests that immunosuppressive drugs in CTD-related ILDs are capable of benefiting a significant proportion of patients, particularly those with certain histological patterns of disease. This review will essentially focus on CTD-associated ILD and will compare aspects of clinical presentation and management to those of IPF.
American Thoracic Society.Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. American Thoracic Society (ATS), and the European Respiratory Society (ERS). Am J Respir Crit Care Med2000; 161: 646-664.
2.
Flaherty KR, Thwaite EL, Kazerooni EA, Gross BH, Toews GB, Colby TV, et al. Radiological versus histological diagnosis in UIP and NSIP: survival implications. Thorax2003; 58: 143-148.
3.
Hunninghake GW, Zimmerman MB, Schwartz DA, King TEJ, Lynch J., Hegele R., et al. Utility of a lung biopsy for the diagnosis of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med2001; 164: 193-196.
4.
Raghu G., Mageto YN, Lockhart D., Schmidt RA, Wood DE and Godwin, JDThe accuracy of the clinical diagnosis of new-onset idiopathic pulmonary fibrosis and other interstitial lung disease: A prospective study. Chest1999; 116: 1168-1174.
5.
Behr J., Vogelmeier C., Beinert T., Meurer M., Krombach F., König G., and Fruhmann G.Bronchoalveolar lavage for evaluation and management of scleroderma disease of the lung. Am J Respir Crit Care Med1996 ; 154: 400-406.
6.
Goh NSL , Veeraraghavan S., Desai SR, Cramer D., Hansell DM, Denton CP, et al. Bronchoalveolar lavage cellular profiles in patients with systemic sclerosis-associated interstitial lung disease are not predictive of disease progression. Arthritis Rheum2007; 56: 2005-2012.
7.
Goh NSL , Desai SR, Veeraraghavan S., Hansell DM, Copley SJ, Maher TM, et al. Interstitial lung disease in systemic sclerosis: a simple staging system. Am J Respir Crit Care Med2008; 177: 1248-1254.
8.
American Thoracic Society/European Respiratory and Society.International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonia. Am J Respir Crit Care Med2002; 165: 277-304.
9.
Harrison NK, Myers AR, Corrin B., Soosay G., Dewar A., Black CM, et al. Structural features of interstitial lung disease in systemic sclerosis. Am Rev Respir Dis1991; 144: 706-713.
10.
Lee H., Kim DS, Yoo B., Seo JB, Rho J., Colby TV, et al. Histopathologic pattern and clinical features of rheumatoid arthritis-associated interstitial lung disease. Chest2005; 127: 2019-2027.
11.
Tanaka N., Kim JS, Newell JD, Brown KK, Cool CD, Meehan R., et al. Rheumatoid arthritis-related lung diseases: CT findings. Radiology2004; 232: 81-91.
12.
Wells AU, Cullinan P., Hansell DM, Rubens MB, Black CM, Newman-Taylor AJet al. Fibrosing alveolitis associated with systemic sclerosis has a better prognosis than lone cryptogenic fibrosing alveolitis. Am J Respir Crit Care Med1994; 149: 1583-1590.
13.
Renzoni E., Rottoli P., Coviello G., Perari MG, Galeazzi M., and Vagliasindi M.Clinical, laboratory and radiological findings in pulmonary fibrosis with and without connective tissue disease. Clin Rheumatol1997; 16: 570-577.
14.
Park JH, Kim DS, Park I., Jang SJ, Kitaichi M., Nicholson AG, et al. Prognosis of fibrotic interstitial pneumonia: idiopathic versus collagen vascular disease-related subtypes. Am J Respir Crit Care Med2007; 175: 705-711.
15.
Parambil JG , Myers JL, and Ryu JHDiffuse alveolar damage: uncommon manifestation of pulmonary involvement in patients with connective tissue diseases. Chest2006; 130: 553-558.
16.
Muir TE, Tazelaar HD, Colby TV, and Myers JLOrganizing diffuse alveolar damage associated with progressive systemic sclerosis . Mayo Clin Proc1997; 72: 639-642.
17.
Phillips K. , Byrne-Dugan C., Batterson E., and Seibold JRRapidly progressive fatal interstitial lung disease in a patient with systemic sclerosis. Nat Rev Rheumatol . 2009; 5: 225-228.
18.
Parambil JG , Myers JL, Lindell RM, Matteson EL, and Ryu JHInterstitial lung disease in primary Sjögren syndrome. Chest2006 ; 130: 1489-1495.
19.
Desai SR, Veeraraghavan S., Hansell DM, Nikolakopolou A., Goh NSL, Nicholson AG, et al. CT features of lung disease in patients with systemic sclerosis: comparison with idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia. Radiology2004; 232: 560-567.
20.
Nicholson AG , Colby TV, du Bois RM, Hansell DM, and Wells AUThe prognostic significance of the histologic pattern of interstitial pneumonia in patients presenting with the clinical entity of cryptogenic fibrosing alveolitis. Am J Respir Crit Care Med2000; 162: 2213-2217.
21.
Bouros D., Wells AU, Nicholson AG, Colby TV, Polychronopoulos V., Pantelidis P., et al. Histopathologic subsets of fibrosing alveolitis in patients with systemic sclerosis and their relationship to outcome. Am J Respir Crit Care Med2002; 165: 1581-1586.
22.
Flaherty KR , Colby TV, Travis WD, Toews GB, Mum-ford J., Murray S., et al. Fibroblastic foci in usual interstitial pneumonia: idiopathic versus collagen vascular disease. Am J Respir Crit Care Med2003; 167: 1410-1415.
23.
Nagao T., Nagai S., Kitaichi M., Hayashi M., Shigematsu M., Tsutsumi T., et al. Usual interstitial pneumonia: idiopathic pulmonary fibrosis versus collagen vascular diseases. Respiration2001; 68: 151-159.
24.
Song JW, Do K., Kim M., Jang SJ, Colby TV, and Kim DSPathologic and radiologic differences between idiopathic and collagen vascular disease-related usual interstitial pneumonia. Chest2009; 136: 23-30.
25.
Wells AU, Hansell DM, Rubens MB, King AD, Cramer D., Black CM et al. Fibrosing alveolitis in systemic sclerosis: indices of lung function in relation to extent of disease on computed tomography. Arthritis Rheum1997; 40: 1229-1236.
26.
Wells AUPulmonary function tests in connective tissue disease. Semin Respir Crit Care Med2007; 28: 379-388.
27.
Reveille JD and Solomon DHEvidence-based guidelines for the use of immunologic tests: anticentromere, Scl-70, and nucleolar antibodies . Arthritis Rheum2003; 49: 399-412.
28.
Laing TJ, Gillespie BW, Toth MB, Mayes MD, Gallavan RHJ, Burns CJ, et al. Racial differences in scleroderma among women in Michigan. Arthritis Rheum1997; 40: 734-742.
29.
Steen VDClinical manifestations of systemic sclerosis. Semin Cutan Med Surg1998; 17: 48-54.
30.
Steen VD, Conte C., Owens GR, and Medsger TAJ.Severe restrictive lung disease in systemic sclerosis. Arthritis Rheum1994; 37: 1283-1289.
31.
Steen VD, Powell DL, and Medsger TAJClinical correlations and prognosis based on serum autoantibodies in patients with systemic sclerosis. Arthritis Rheum1988 ; 31: 196-203.
32.
Wells AU, Hansell DM, Harrison NK, Lawrence R., Black CM, and du Bois RMClearance of inhaled 99mTc-DTPA predicts the clinical course of fibrosing alveolitis. Eur Respir J1993; 6: 797-802.
33.
Takahashi H. , Kuroki Y., Tanaka H., Saito T., Kurokawa K., Chiba H., et al. Serum levels of surfactant proteins A and D are useful biomarkers for interstitial lung disease in patients with progressive systemic sclerosis . Am J Respir Crit Care Med2000; 162: 258-263.
34.
Yanaba K., Hasegawa M., Takehara K., and Sato S.Comparative study of serum surfactant protein-D and KL-6 concentrations in patients with systemic sclerosis as markers for monitoring the activity of pulmonary fibrosis. J Rheumatol2004; 31: 1112-1120.
35.
Tashkin DP, Elashoff R., Clements PJ, Goldin J., Roth MD, Furst DE, et al. Cyclophosphamide versus placebo in scleroderma lung disease . N Engl J Med2006; 354: 2655-2666.
36.
Hoyles RK, Ellis RW, Wellsbury J., Lees B., Newlands P., Goh NSL, et al. A multicenter, prospective, randomized, double-blind, placebo-controlled trial of and intravenous cyclophosphamide followed by oral azathioprine for the treatment of pulmonary fibrosis in scleroderma. Arthritis Rheum2006; 54: 3962-3970.
37.
Haubitz M., Schellong S., Göbel U., Schurek HJ, Schaumann D., Koch KM et al. Intravenous pulse administration of cyclophosphamide versus daily oral treatment in patients with antineutrophil cytoplasmic antibody-associated vasculitis and renal involvement: a prospective, randomized study. Arthritis Rheum1998; 41: 1835-1844.
38.
Tashkin DP, Elashoff R., Clements PJ, Roth MD, Furst DE, Silver RM, et al. Effects of 1-year treatment with cyclophosphamide on outcomes at 2 years in scleroderma lung disease. Am J Respir Crit Care Med2007; 176, 1026-1034.
39.
Zamora AC, Wolters PJ, Collard HR, Connolly MK, Elicker BM, Webb WR, et al. Use of mycophenolate mofetil to treat scleroderma-associated interstitial lung disease. Respir Med2008 ; 102, 150-155.
40.
Gerbino AJ, Goss CH, and Molitor JAEffect of mycophenolate mofetil on pulmonary function in scleroderma-associated interstitial lung disease. Chest2008; 133: 455-460.
41.
Nihtyanova SI, Brough GM, Black CM, and Denton CPMycophenolate mofetil in diffuse cutaneous systemic sclerosis-a retrospective analysis. Rheumatology (Oxford) 2007; 46, 442-445.
42.
Hachulla E. , de Groote P., Gressin V., Sibilia J., Diot E., Carpentier P., et al. The three-year incidence of pulmonary arterial hypertension associated with systemic sclerosis in a multicenter nationwide longitudinal study in France . Arthritis Rheum2009; 60: 1831-1839.
43.
Steen V., and Medsger TAJ.Predictors of isolated pulmonary hypertension in patients with systemic sclerosis and limited cutaneous involvement. Arthritis Rheum2003; 48, 516-522.
44.
Ungerer RG, Tashkin DP, Furst D., Clements PJ, Gong HJ, Bein M., et al. Prevalence and clinical correlates of pulmonary arterial hypertension in progressive systemic sclerosis. Am J Med1983; 75: 65-74.
45.
Kawut SM, Taichman DB, Archer-Chicko CL, Palevsky HI, and Kimmel SEHemodynamics and survival in patients with pulmonary arterial hypertension related to systemic sclerosis. Chest2003; 123: 344-350.
46.
Fisher MR, Mathai SC, Champion HC, Girgis RE, Housten-Harris T., Hummers L., et al. Clinical differences between idiopathic and scleroderma-related pulmonary hypertension. Arthritis Rheum2006 ; 54: 3043-3050.
47.
Hunzelmann N., Genth E., Krieg T., Lehmacher W., Melchers I., Meurer M., et al. The registry of the German Network for Systemic Scleroderma: frequency of disease subsets and patterns of organ involvement. Rheumatology ( Oxford) 2008; 47: 1185-1192.
48.
Channick RN , Simonneau G., Sitbon O., Robbins IM, Frost A., Tapson VF, et al. Effects of the dual endothelin-receptor antagonist bosentan in patients with pulmonary hypertension: a randomised placebo-controlled study. Lancet2001; 358: 1119-1123.
49.
Rubin LJ, Badesch DB, Barst RJ, Galie N., Black CM, Keogh A., et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med2002; 346: 896-903.
50.
Galiè N., Olschewski H., Oudiz RJ, Torres F., Frost A., Ghofrani HA, et al. Ambrisentan for the treatment of pulmonary arterial hypertension: results of the ambrisentan in pulmonary arterial hypertension, randomized, double-blind, placebo-controlled, multicenter, efficacy (ARIES) study 1 and 2. Circulation2008; 117: 3010-3019.
51.
Galiè N., Ghofrani HA, Torbicki A., Barst RJ, Rubin LJ, Badesch D., et al. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med2005; 353: 2148-2157.
52.
Launay D., Hachulla E., Hatron PY, Goullard L., Onimus T., Robin S., et al. Aerosolized iloprost in CREST syndrome related pulmonary hypertension . J Rheumatol2001; 28: 2252-2256.
53.
Badesch DB, Tapson VF, McGoon MD, Brundage BH, Rubin LJ, Wigley FM, et al. Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized, controlled trial. Ann Intern Med2000; 132: 425-434.
54.
Joglekar A. , Tsai FS, McCloskey DA, Wilson JE, Seibold JR, and Riley DJBosentan in pulmonary arterial hypertension secondary to scleroderma. J Rheumatol2006 ; 33: 61-68.
55.
Williams MH , Das C., Handler CE, Akram MR, Davar J., Denton CP, et al. Systemic sclerosis associated pulmonary hypertension: improved survival in the current era. Heart2006; 92: 926-932.
56.
Koh ET, Lee P., Gladman DD, and Abu-Shakra M.Pulmonary hypertension in systemic sclerosis: an analysis of 17 patients . Br J Rheumatol1996; 35: 989-993.
57.
Mukerjee D. , St George D., Coleiro B., Knight C., Denton CP, Davar J., et al. Prevalence and outcome in systemic sclerosis associated pulmonary arterial hypertension: application of a registry approach. Ann Rheum Dis2003; 62: 1088-1093.
58.
Launay D., Sitbon O., Le Pavec J., Savale L., Tchérakian C., Yaïci A., et al. Long-term outcome of systemic sclerosis-associated pulmonary arterial hypertension treated with bosentan as first-line monotherapy followed or not by the addition of prostanoids or sildenafil. Rheumatology (Oxford) 2010; 49: 490-500.
59.
Dorfmüller P., Humbert M., Perros F., Sanchez O., Simonneau G., Müller K., et al. Fibrous remodeling of the pulmonary venous system in pulmonary arterial hypertension associated with connective tissue diseases. Hum Pathol2007; 38: 893-902.
60.
Kahan A., Devaux JY, Amor B., Menkès CJ, Weber S., Nitenberg A., et al. Nifedipine and thallium-201 myocardial perfusion in progressive systemic sclerosis. N Engl J Med1986; 314: 1397-1402.
61.
Fernandes F. , Ramires FJA, Arteaga E., Ianni BM, Bonfá Esdo, and Mady C.Cardiac remodeling in patients with systemic sclerosis with no signs or symptoms of heart failure: an endomyocardial biopsy study. J Card Fail2003; 9: 311-317.
62.
Allanore Y. , Borderie D., Avouac J., Zerkak D., Meune C., Hachulla E., et al. High N-terminal pro-brain natriuretic peptide levels and low diffusing capacity for carbon monoxide as independent predictors of the occurrence of precapillary pulmonary arterial hypertension in patients with systemic sclerosis. Arthritis Rheum2008; 58: 284-291.
63.
Korkmaz C., Us T., Kaşifoğlu T., and Akgün Y.Anti-cyclic citrullinated peptide (CCP) antibodies in patients with long-standing rheumatoid arthritis and their relationship with extra-articular manifestations . Clin Biochem2006; 39: 961-965.
64.
Nielen MMJ , van Schaardenburg D., Reesink HW, van de Stadt RJ, van der Horst-Bruinsma IE, de Koning Mhmt, et al. Specific autoantibodies precede the symptoms of rheumatoid arthritis: a study of serial measurements in blood donors. Arthritis Rheum2004; 50: 380-386.
65.
Oliver JE and Silman AJRisk factors for the development of rheumatoid arthritis. Scand J Rheumatol2006; 35: 169-174.
66.
Gizinski AM , Mascolo M., Loucks JL, Kervitsky A., Meehan RT, Brown KK, et al. Rheumatoid arthritis (RA)-specific autoantibodies in patients with interstitial lung disease and absence of clinically apparent articular RA. Clin Rheumatol2009 ; 28: 611-613.
67.
Sihvonen S. , Korpela M., Laippala P., Mustonen J., and Pasternack A.Death rates and causes of death in patients with rheumatoid arthritis: a population-based study. Scand J Rheumatol2004; 33: 221-227.
68.
Tansey D., Wells AU, Colby TV, Ip S., Nikolakoupolou A., du Bois RM, et al. Variations in histological patterns of interstitial pneumonia between connective tissue disorders and their relationship to prognosis. Histopathology2004; 44: 585-596.
69.
Hyland RH, Gordon DA, Broder I., Davies GM, Russell ML, Hutcheon MA, et al. A systematic controlled study of pulmonary abnormalities in rheumatoid arthritis. J Rheumatol1983; 10: 395-405.
70.
Jurik AG, Davidsen D., and Graudal H.Prevalence of pulmonary involvement in rheumatoid arthritis and its relationship to some characteristics of the patients. A radiological and clinical study . Scand J Rheumatol1982; 11: 217-224.
71.
Gabbay E., Tarala R., Will R., Carroll G., Adler B., Cameron D., and Lake FRInterstitial lung disease in recent onset rheumatoid arthritis. Am J Respir Crit Care Med1997; 156: 528-535.
72.
Saag KG, Cerhan JR, Kolluri S., Ohashi K., Hunninghake GW, and Schwartz DACigarette smoking and rheumatoid arthritis severity. Ann Rheum Dis1997; 56: 463-469.
73.
Kim EJ, Collard HR, and King TEJRheumatoid arthritis-associated interstitial lung disease: the relevance of histopathologic and radiographic pattern. Chest2009; 136: 1397-1405.
74.
Kim EJ, Elicker BM, Maldonado F., Webb WR, Ryu JH, Van Uden JH, et al. Usual interstitial pneumonia in rheumatoid arthritis-associated interstitial lung disease. Eur Respir J2010 ;35: 1322-1328.
75.
Barrera P., Laan RF, van Riel PL, Dekhuijzen PN, Boerbooms AM, and van de Putte LBMethotrexate-related pulmonary complications in rheumatoid arthritis . Ann Rheum Dis1994; 53: 434-439.
76.
Golden MR, Katz RS, Balk RA, and Golden HEThe relationship of preexisting lung disease to the development of methotrexate pneumonitis in patients with rheumatoid arthritis. J Rheumatol1995; 22: 1043-1047.
77.
Alarcόn GS, Kremer JM, Macaluso M., Weinblatt ME, Cannon GW, Palmer WR, The relationship of preexisting lung disease to the development of methotrexate pneumonitis in patients with rheumatoid arthritis Risk factors for methotrexate-induced lung injury in patients with rheumatoid arthritis. A multicenter, case-control study. Methotrexate-Lung Study Group. Ann Intern Med1997; 127: 356-364.
78.
Arakawa H., Yamasaki M., Kurihara Y., Yamada H., and Nakajima Y.Methotrexate-induced pulmonary injury: serial CT findings. J Thorac Imaging2003; 18: 231-236.
79.
Sakai F., Noma S., Kurihara Y., Yamada H., Azuma A., Kudoh S., et al. Leflunomide-related lung injury in patients with rheumatoid arthritis: imaging features. Mod Rheumatol2005; 15: 173-179.
80.
Savage RL, Highton J., Boyd IW, and Chapman P.Pneumonitis associated with leflunomide: a profile of New Zealand andAustralian reports. Intern Med J2006 ; 36: 162-169.
81.
Wardwell NRJ , Miller R., and Ware LBPulmonary alveolar proteinosis associated with a disease-modifying antirheumatoid arthritis drug . Respirology2006; 11: 663-665.
82.
Carloni A., Piciucchi S., Giannakakis K., Nori G., Zobel BB, and Poletti V.Diffuse alveolar hemorrhage after leflunomide therapy in a patient with rheumatoid arthritis. J Thorac Imaging2008; 23: 57-59.
83.
Scott DL, Bradby GV, Aitman TJ, Zaphiropoulos GC, and Hawkins CFRelationship of gold and penicillamine therapy to diffuse interstitial lung disease. Ann Rheum Dis1981; 40: 136-141.
84.
Parry SD, Barbatzas C., Peel ET, and Barton JRSulphasalazine and lung toxicityEur Respir J2002; 19: 756-764.
85.
Klein HP, Rupp M., and Thiele-Fischbach A.[Drug-induced alveolitis caused by salazosulfapyridine] . Dtsch Med Wochenschr1988; 113: 1239-1241.
86.
Hagiwara K. , Sato T., Takagi-Kobayashi S., Hasegawa S., Shigihara N., and Akiyama O.Acute exacerbation of preexisting interstitial lung disease after administration of etanercept for rheumatoid arthritis. J Rheumatol2007 ; 34: 1151-1154.
87.
Thavarajah K., Wu P., Rhew EJ, Yeldandi AK, and Kamp DWPulmonary complications of tumor necrosis factor-targeted therapy. Respir Med2009; 103: 661-669.
88.
Kimby E.Tolerability and safety of rituximab (MabThera). Cancer Treat Rev2005; 31: 456-473.
89.
Hainsworth JDSafety of rituximab in the treatment of B cell malignancies: implications for rheumatoid arthritis. Arthritis Res Ther2003; 5 Suppl4: S12-6.
90.
Soubrier M. , Jeannin G., Kemeny JL, Tournadre A., Caillot N., Caillaud D., et al. Organizing pneumonia after rituximab therapy: two cases. Joint Bone Spine2008; 75, 362-365.
91.
Dalakas MC and Hohlfeld R.Polymyositis and dermatomyositis. Lancet2003 ; 362: 971-982.
92.
Bohan A. and Peter JBPolymyositis and dermatomyositis (first of two parts). N Engl J Med1975; 292: 344-347.
93.
Bohan A. and Peter JBPolymyositis and dermatomyositis (second of two parts). N Engl J Med1975; 292: 403-407.
94.
Troyanov Y. , Targoff IN, Tremblay J., Goulet J., Raymond Y., and Senécal J.Novel classification of idiopathic inflammatory myopathies based on overlap syndrome features and autoantibodies: analysis of 100 FrenchCanadian patients. Medicine (Baltimore) 2005 ; 84: 231-249.
95.
Krain L.Dermatomyositis in six patients without initial muscle involvement . Arch Dermatol1975; 111: 241-245.
96.
Caproni M., Cardinali C., Parodi A., Giomi B., Papini M., Vaccaro M., et al. Amyopathic dermatomyositis: a review by the Italian Group of Immunodermatology. Arch Dermatol2002; 138: 23-27.
97.
Crestani B.The respiratory system in connective tissue disorders. Allergy2005; 60: 715-734.
98.
Fathi M., Lundberg IE, and Tornling G.Pulmonary complications of polymyositis and dermatomyositis. Semin Respir Crit Care Med2007; 28: 451-458.
99.
Schwarz MIThe lung in polymyositis. Clin Chest Med1998 ; 19: 701-12, viii.
100.
Marie I., Hachulla E., Chérin P., Dominique S., Hatron P., Hellot M., et al. Interstitial lung disease in polymyositis and dermatomyositis . Arthritis Rheum. 2002; 47: 614-622.
101.
Dankό K. , Ponyi A., Constantin T., Borgulya G., and Szegedi G.Long-term survival of patients with idiopathic inflammatory myopathies according to clinical features: a longitudinal study of 162 cases. Medicine ( Baltimore) 2004; 83: 35-42.
102.
Chen I., Jan Wu Y., Lin C., Fan K., Luo S., Ho H., et al. Interstitial lung disease in polymyositis and dermatomyositis . Clin Rheumatol2009; 28: 639-646.
103.
Marie I., Hachulla E., Hatron PY, Hellot MF, Levesque H., Devulder B., and Courtois H.Polymyositis and dermatomyositis: short term and longterm outcome, and predictive factors of prognosis. J Rheumatol2001; 28: 2230-2237.
104.
Lakhanpal S., Lie JT, Conn DL, and Martin WJ 2. Pulmonary disease in polymyositis/dermatomyositis: a clinicopathological analysis of 65 autopsy cases. Ann Rheum Dis1987; 46: 23-29.
105.
Marie I., Hatron PY, Hachulla E., Wallaert B., Michon-Pasturel U., and Devulder B.Pulmonary involvement in polymyositis and in dermatomyositis. J Rheumatol1998; 25: 1336-1343.
106.
Dickey BF and Myers ARPulmonary disease in polymyositis/dermatomyositis. Semin Arthritis Rheum1984; 14: 60-76.
107.
Fathi M., Dastmalchi M., Rasmussen E., Lundberg IE, and Tornling G.Interstitial lung disease, a common manifestation of newly diagnosed polymyositis and dermatomyositis. Ann Rheum Dis2004; 63: 297-301.
108.
Cottin V., Thivolet-Béjui F., Reynaud-Gaubert M., Cadranel J., Delaval P., Ternamian PJ, et al. Interstitial lung disease in amyopathic dermatomyositis, dermatomyositis and polymyositis. Eur Respir J2003; 22: 245-250.
109.
Selva-O’Callaghan A., Labrador-Horrillo M., Muñoz-Gall X., Martínez-Gomez X., Majό-Masferrer J., Solans-Laque R., et al. Polymyositis/dermatomyositis-associated lung disease: analysis of a series of 81 patients. Lupus2005; 14: 534-542.
110.
Schnabel A. , Reuter M., Biederer J., Richter C., and Gross WLInterstitial lung disease in polymyositis and dermatomyositis: clinical course and response to treatment. Semin Arthritis Rheum2003 ; 32: 273-284.
111.
Won Huh J., Soon Kim D., Keun Lee C., Yoo B., Bum Seo J., Kitaichi M., et al. Two distinct clinical types of interstitial lung disease associated with polymyositisdermatomyositis . Respir Med2007; 101: 1761-1769.
112.
Fathi M., Vikgren J., Boijsen M., Tylen U., Jorfeldt L., Tornling G., and Lundberg IEInterstitial lung disease in polymyositis and dermatomyositis: longitudinal evaluation by pulmonary function and radiology. Arthritis Rheum2008; 59: 677-685.
113.
Tazelaar HD , Viggiano RW, Pickersgill J., and Colby TVInterstitial lung disease in polymyositis and dermatomyositis. Clinical features and prognosis as correlated with histologic findings. Am Rev Respir Dis1990; 141: 727-733.
114.
Bonnefoy O. , Ferretti G., Calaque O., Coulomb M., Begueret H., Beylot-Barry M., et al. Serial chest CT findings in interstitial lung disease associated with polymyositis-dermatomyositis . Eur J Radiol2004; 49: 235-244.
115.
Douglas WW , Tazelaar HD, Hartman TE, Hartman RP, Decker PA, Schroeder DR, et al. Polymyositis-dermatomyositis-associated interstitial lung disease. Am J Respir Crit Care Med2001; 164: 1182-1185.
116.
Devaraj A. , Wells AU, and Hansell DMComputed tomographic imaging in connective tissue diseases. Semin Respir Crit Care Med2007; 28: 389-397.
117.
Akira M., Hara H., and Sakatani M.Interstitial lung disease in association with polymyositisdermatomyositis: long-term follow-up CT evaluation in seven patients. Radiology1999; 210: 333-338.
118.
Ikezoe J., Johkoh T., Kohno N., Takeuchi N., Ichikado K., and Nakamura H.High-resolution CT findings of lung disease in patients with polymyositis and dermatomyositis. J Thorac Imaging1996 ; 11: 250-259.
119.
Mino M., Noma S., Taguchi Y., Tomii K., Kohri Y., and Oida K.Pulmonary involvement in polymyositis and dermatomyositis: sequential evaluation with CT. AJR Am J Roentgenol1997 ; 169: 83-87.
120.
Friedman AW , Targoff IN, and Arnett FCInterstitial lung disease with autoantibodies against aminoacyltRNA synthetases in the absence of clinically apparent myositis . Semin Arthritis Rheum1996; 26: 459-467.
121.
Imbert-Masseau A., Hamidou M., Agard C., Grolleau JY, and Chérin P.Antisynthetase syndrome . Joint Bone Spine2003; 70: 161-168.
122.
Love LA, Leff RL, Fraser DD, Targoff IN, Dalakas M., Plotz PH, et al. A new approach to the classification of idiopathic inflammatory myopathy: myositis-specific autoantibodies define useful homogeneous patient groups. Medicine (Baltimore) 1991; 70: 360-374.
123.
Evans J.Antinuclear antibody testing in systemic autoimmune disease. Clin Chest Med1998; 19: 613-625, vii.
Tillie-Leblond I., Wislez M., Valeyre D., Crestani B., Rabbat A., Israel-Biet D., et al. Interstitial lung disease and anti-Jo-1 antibodes: difference between acute and gradual onset. Thorax2008; 63: 53-59.
126.
Bandoh S., Fujita J., Ohtsuki Y., Ueda Y., Hojo S., Tokuda M., et al. Sequential changes of KL-6 in sera of patients with interstitial pneumonia associated with polymyositis/dermatomyositis. Ann Rheum Dis2000; 59: 257-262.
127.
Kumánovics G., Minier T., Radics J., Pálinkás L., Berki T., and Czirják L.Comprehensive investigation of novel serum markers of pulmonary fibrosis associated with systemic sclerosis and dermato/polymyositis . Clin Exp Rheumatol2008; 26: 414-420.
128.
Ihn H. , Asano Y., Kubo M., Yamane K., Jinnin M., Yazawa N., et al. Clinical significance of serum surfactant protein D (SP-D) in patients with polymyositis/dermatomyositis: correlation with interstitial lung disease. Rheumatology ( Oxford) 2002; 41: 1268-1272.
129.
Fujita J., Dobashi N., Tokuda M., Bandoh S., Ohtsuki Y., Yamadori I., et al. Elevation of cytokeratin 19 fragment in patients with interstitial pneumonia associated with polymyositis/dermatomyositis. J Rheumatol1999; 26: 2377-2382.
130.
Martin L., Chalmers IM, Dhingra S., McCarthy D., and Hunter T.Measurements of maximum respiratory pressures in polymyositis and dermatomyositis . J Rheumatol1985; 12: 104-107.
131.
Dauriat G. , Stern J., Similowski T., Herson S., Belmatoug N., Marceau A., et al. Acute respiratory failure due to diaphragmatic weakness revealing a polymyositis . Eur J Intern Med2002; 13: 203-205.
132.
Hill CL, Zhang Y., Sigurgeirsson B., Pukkala E., Mellemkjaer L., Airio A., et al. Frequency of specific cancer types in dermatomyositis and polymyositis: a population-based study. Lancet2001; 357: 96-100.
133.
Kameda H. and Takeuchi T.Recent advances in the treatment of interstitial lung disease in patients with polymyositis/dermatomyositis. Endocr Metab Immune Disord Drug Targets2006; 6: 409-415.
134.
Maccioni FJ and Colebatch HJManagement of fibrosing alveolitis with polymyositis dermatomyositis. Aust N Z J Med1990; 20: 806-810.
135.
Sem M. , Molberg O., Lund MB, and Gran JTRituximab treatment of the anti-synthetase syndrome: a retrospective case series. Rheumatology ( Oxford) 2009; 48: 968-971.
136.
Lambotte O. , Kotb R., Maigne G., Blanc F., Goujard C., and Delfraissy JFEfficacy of rituximab in refractory polymyositis. J Rheumatol2005 ; 32: 1369-1370.
137.
Kristofova B., Oetterova M., Valocikova I., Macejova Z., Pidanicova A., Firment J., et al. Successful therapy with intravenous immunoglobulin in the management of polymyositis . Bratisl Lek Listy2008; 109: 412-413.
138.
Vitali C., Bombardieri S., Jonsson R., Moutsopoulos HM, Alexander EL, Carsons SE, et al. Classification criteria for Sjögren’s syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis2002; 61: 554-558.
139.
Alexander EL, Provost TT, Stevens MB, and Alexander GENeurologic complications of primary Sjögren’s syndrome. Medicine (Baltimore) 1982; 61: 247-257.
140.
Salliot C. , Mouthon L., Ardizzone M., Sibilia J., Guillevin L., Gottenberg J., et al. Sjögren’s syndrome is associated with and not secondary to systemic sclerosis. Rheumatology (Oxford) 2007; 46: 321-326.
141.
Manoussakis MN, Georgopoulou C., Zintzaras E., Spyropoulou M., Stavropoulou A., Skopouli FN, et al. Sjögren’s syndrome associated with systemic lupus erythematosus: clinical and laboratory profiles and comparison with primary Sjögren’s syndrome. Arthritis Rheum2004; 50: 882-891.
142.
Ramos-Casals M., Brito-Zerόn P., and Font J.The overlap of Sjögren’s syndrome with other systemic autoimmune diseases. Semin Arthritis Rheum2007; 36: 246-255.
143.
Carine S., Gottenberg JE, Bengoufa D., Desmoulins F., Miceli-Richard C., and Mariette X.Anticentromere antibodies identify patients with Sjögren’s syndrome and autoimmune overlap syndrome. J Rheumatol2007; 34: 2253-2258.
144.
Matsuyama N., Ashizawa K., Okimoto T., Kadota J., Amano H., and Hayashi K.Pulmonary lesions associated with Sjögren’s syndrome.radiographic and CT findings . Br J Radiol2003; 76: 880-884.
145.
Uffmann M. , Kiener HP, Bankier AA, Baldt MM, Zontsich T., and Herold CJLung manifestation in asymptomatic patients with primary Sjögren syndrome: assessment with high resolution CT and pulmonary function tests. J Thorac Imaging2001; 16: 282-289.
146.
Franquet T. , Giménez A., Monill JM, Díaz C., and Geli C.Primary Sjögren’s syndrome and associated lung disease: CT findings in 50 patients. AJR Am J Roentgenol1997; 169: 655-658.
147.
Lohrmann C. , Uhl M., Warnatz K., Ghanem N., Kotter E., Schaefer O., et al. High-resolution CT imaging of the lung for patients with primary Sjögren’s syndrome . Eur J Radiol2004; 52: 137-143.
148.
Koyama M., Johkoh T., Honda O., Mihara N., Kozuka T., Tomiyama N., et al. Pulmonary involvement in primary Sjögren’s syndrome: spectrum of pulmonary abnormalities and computed tomography findings in 60 patients. J Thorac Imaging2001; 16: 290-296.
149.
Davidson BK , Kelly CA, and Griffiths IDTen year follow up of pulmonary function in patients with primary Sjögren’s syndrome. Ann Rheum Dis2000; 59: 709-712.
150.
Kelly CA, Foster H., Pal B., Gardiner P., Malcolm AJ, Charles P., et al. Primary Sjögren’s syndrome in north east England-a longitudinal study. Br J Rheumatol1991; 30: 437-442.
151.
Constantopoulos SH, Papadimitriou CS, and Moutsopoulos HMRespiratory manifestations in primary Sjögren’s syndrome. A clinical, functional, and histologic study. Chest1985; 88: 226-229.
152.
Lahdensuo A. and Korpela M.Pulmonary findings in patients with primary Sjögren’s syndrome. Chest1995; 108: 316-319.
153.
Papathanasiou MP, Constantopoulos SH, Tsampoulas C., Drosos AA, and Moutsopoulos HMReappraisal of respiratory abnormalities in primary and secondary Sjögren’s syndrome. A controlled study. Chest1986; 90: 370-374.
154.
Deheinzelin D., Capelozzi VL, Kairalla RA, Barbas Filho JV, Saldiva PH, and de Carvalho CRInterstitial lung disease in primary Sjögren’s syndrome. Clinical-pathological evaluation and response to treatment. Am J Respir Crit Care Med1996; 154: 794-799.
155.
Ramos-Casals M., Solans R., Rosas J., Camps MT, Gil A., Del Pino-Montes J., et al. Primary Sjögren syndrome in Spain: clinical and immunologic expression in 1010 atients. Medicine (Baltimore) 2008; 87: 210-219.
156.
Cain HC, Noble PW, and Matthay RAPulmonary manifestations of Sjögren’s syndrome. Clin Chest Med1998; 19: 687-99, viii.
157.
Launay D., Hachulla E., Hatron P., Jais X., Simonneau G., and Humbert M.Pulmonary arterial hypertension: a rare complication of primary Sjögren syndrome: report of 9 new cases and review of the literature. Medicine (Baltimore) 2007; 86: 299-315.
158.
Chen H., Lai S., Kwang W., Liu J., Chen C., and Huang D.Middle lobe syndrome as the pulmonary manifestation of primary Sjögren’s syndrome. Med J Aust2006; 184: 294-295.
159.
Tavoni A., Vitali C., Cirigliano G., Frigelli S., Stampacchia G., and Bombardieri S.Shrinking lung in primary Sjögren’s syndrome. Arthritis Rheum1999; 42: 2249-2250.
160.
Ito I. , Nagai S., Kitaichi M., Nicholson AG, Johkoh T., Noma S., et al. Pulmonary manifestations of primary Sjögren’s syndrome: a clinical, radiologic, and pathologic study . Am J Respir Crit Care Med2005; 171: 632-638.
161.
Papiris SA , Maniati M., Constantopoulos SH, Roussos C., Moutsopoulos HM, and Skopouli FNLung involvement in primary Sjögren’s syndrome is mainly related to the small airway disease. Ann Rheum Dis1999; 58: 61-64.
162.
Mialon P., Barthélémy L., Sébert P., Le Hénaff C., Sarni D., Pennec YL, et al. A longitudinal study of lung impairment in patients with primary Sjögren’s syndrome. Clin Exp Rheumatol1997; 15: 349-354.
163.
Bloch KJ, Buchanan WW, Wohl MJ, and Bunim JJSjögren’s syndrome. A clinical, pathological, and serological study of sixty-two cases. Medicine (Baltimore ) 1965; 44: 187-231.
164.
Salaffi F. , Manganelli P., Carotti M., Baldelli S., Blasetti P., Subiaco S., et al. A longitudinal study of pulmonary involvement in primary Sjögren’s syndrome: relationship between alveolitis and subsequent lung changes on high-resolution computed tomography . Br J Rheumatol1998; 37: 263-269.
165.
Yamadori I. , Fujita J., Bandoh S., Tokuda M., Tanimoto Y., Kataoka M., et al. Nonspecific interstitial pneumonia as pulmonary involvement of primary Sjögren’s syndrome. Rheumatol Int2002; 22: 89-92.
166.
Shi J. , Liu H., Xu W., Feng R., Zhang Z., Tian X., et al. Pulmonary manifestations of Sjögren’s Syndrome. Respiration2009; 78: 377-386.
167.
Bradley B. , Branley HM, Egan JJ, Greaves MS, Hansell DM, Harrison NK, et al. Interstitial lung disease guideline: the British Thoracic Society in collaboration with the Thoracic Society of Australia and New Zealand and the Irish Thoracic Society . Thorax2008; 63 Suppl5: v1-58.
168.
Honda O., Johkoh T., Ichikado K., Tomiyama N., Maeda M., Mihara N., et al. Differential diagnosis of lymphocytic interstitial pneumonia and malignant lymphoma on high-resolution CT. AJR Am J Roentgenol1999; 173: 71-74.
169.
Nicholson AG, Colby TV, and Wells AUHistopathological approach to patterns of interstitial pneumonia in patient with connective tissue disorders. Sarcoidosis Vasc Diffuse Lung Dis2002; 19: 10-17.
170.
Wallaert B. , Hatron PY, Grosbois JM, Tonnel AB, Devulder B., and Voisin C.Subclinical pulmonary involvement in collagen-vascular diseases assessed by bronchoalveolar lavage. Relationship between alveolitis and subsequent changes in lung function. Am Rev Respir Dis1986; 133: 574-580.
171.
Hatron PY, Wallaert B., Gosset D., Tonnel AB, Gosselin B., Voisin C., et al. Subclinical lung inflammation in primary Sjögren’s syndrome. Relationship between bronchoalveolar lavage cellular analysis findings and characteristics of the disease. Arthritis Rheum1987; 30: 1226-1231.
172.
Valesini G. , Priori R., Bavoillot D., Osborn J., Danieli MG, Del Papa N., et al. Differential risk of non-Hodgkin’s lymphoma in Italian patients with primary Sjögren’s syndrome. J Rheumatol1997; 24: 2376-2380.
173.
Kassan SS, Thomas TL, Moutsopoulos HM, Hoover R., Kimberly RP, Budman DR, et al. Increased risk of lymphoma in sicca syndromeAnn Intern Med1978; 89: 888-892.
174.
Voulgarelis M., Dafni UG, Isenberg DA, and Moutsopoulos HMMalignant lymphoma in primary Sjögren’s syndrome: a multicenter, retrospective, clinical study by the European Concerted Action on Sjögren’s Syndrome . Arthritis Rheum1999; 42: 1765-1772.
175.
Tonami H., Matoba M., Kuginuki Y., Yokota H., Higashi K., Yamamoto I., et al. Clinical and imaging findings of lymphoma in patients with Sjögren syndrome. J Comput Assist Tomogr2003; 27: 517-524.
176.
Fox R.Sjögren’s syndrome. Lancet2005; 366: 321-331.
177.
Royer B., Cazals-Hatem D., Sibilia J., Agbalika F., Cayuela JM, Soussi T., et al. Lymphomas in patients with Sjögren’s syndrome are marginal zone B-cell neoplasms, arise in diverse extranodal and nodal sites, and are not associated with viruses. Blood1997; 90: 766-775.
178.
Hansen LA, Prakash UB, and Colby TVPulmonary lymphoma in Sjögren’s syndrome. Mayo Clin Proc1989; 64: 920-931.
179.
Voulgarelis M., Tzioufas AG, and Moutsopoulos HMMortality in Sjögren’s syndrome. Clin Exp Rheumatol2008; 26: S66-S71.
180.
Theander E. , Manthorpe R., and Jacobsson LTH.Mortality and causes of death in primary Sjögren’s syndrome: a prospective cohort study. Arthritis Rheum2004; 50: 1262-1269.
181.
Chakravarty EF, Bush TM, Manzi S., Clarke AE, and Ward MMPrevalence of adult systemic lupus erythematosus in California and Pennsylvania in 2000: estimates obtained using hospitalization data. Arthritis Rheum2007; 56: 2092-2094.
182.
Swaak AJ, Nossent JC, and Smeenk RJSystemic lupus erythematosus. Int J Clin Lab Res1992; 22: 190-195.
183.
Cervera R. , Khamashta MA, Font J., Sebastiani GD, Gil A., Lavilla P., et al. Systemic lupus erythematosus: clinical and immunologic patterns of disease expression in a cohort of 1,000 patients . The European Working Party on Systemic Lupus Erythematosus Medicine (Baltimore) 1993; 72: 113-124.
184.
Tan EM , Cohen AS, Fries JF, Masi AT, McShane DJ, Rothfield NF, et al. The 1982 revised criteria for the classification of systemic lupus erythematosus . Arthritis Rheum1982; 25: 1271-1277.
185.
Hochberg MCUpdating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum1997; 40: 1725.
186.
Cervera R. , Khamashta MA, Font J., Sebastiani GD, Gil A., Lavilla P., et al. Morbidity. and mortality in systemic lupus erythematosus during a 5-year period. A multicenter prospective study of 1,000 patients. European Working Party on Systemic Lupus Erythematosus . Medicine (Baltimore) 1999; 78: 167-175.
187.
Bernatsky S., Boivin J., Joseph L., Manzi S., Ginzler E., Gladman DD, et al. Mortality in systemic lupus erythematosus . Arthritis Rheum2006; 54: 2550-2557.
188.
Abu-Shakra M., Urowitz MB, Gladman DD, and Gough J.Mortality studies in systemic lupus erythematosus. Results from a single center. II. Predictor variables for mortality. J Rheumatol1995 ; 22: 1265-1270.
189.
Stoll T., Seifert B., and Isenberg DASLICC/ACR Damage Index is valid, and renal and pulmonary organ scores are predictors of severe outcome in patients with systemic lupus erythematosus . Br J Rheumatol1996; 35: 248-254.
190.
Jiménez S., Cervera R., Font J., and Ingelmo M.The epidemiology of systemic lupus erythematosus . Clin Rev Allergy Immunol2003; 25: 3-12.
191.
Fenlon HM, Doran M., Sant SM, and Breatnach E.High-resolution chest CT in systemic lupus erythematosus. AJR Am J Roentgenol1996; 166: 301-307.
192.
Pego-Reigosa JM, Medeiros DA, and Isenberg DARespiratory manifestations of systemic lupus erythematosus: old and new concepts. Best Pract Res Clin Rheumatol2009; 23: 469-480.
193.
Bertoli AM, Vila LM, Apte M., Fessler BJ, Bastian HM, Reveille JD, and Alarcon GSSystemic lupus erythematosus in a multiethnic US Cohort LUMINA XLVIII: factors predictive of pulmonary damage. Lupus2007 ; 16: 410-417.
194.
Kim JS, Lee KS, Koh EM, Kim SY, Chung MP, and Han J.Thoracic involvement of systemic lupus erythematosus: clinical, pathologic, and radiologic findings. J Comput Assist Tomogr2000; 24: 9-18.
195.
Haupt HM, Moore GW, and Hutchins GMThe lung in systemic lupus erythematosus. Analysis of the pathologic changes in 120 patients. Am J Med1981; 71: 791-798.
196.
Mochizuki T., Aotsuka S., and Satoh T.Clinical and laboratory features of lupus patients with complicating pulmonary disease. Respir Med1999; 93: 95-101.
197.
Todd DJ and Costenbader KHDyspnoea in a young woman with active systemic lupus erythematosus . Lupus2009; 18: 777-784.
198.
Estes D. and Christian CLThe natural history of systemic lupus erythematosus by prospective analysis . Medicine (Baltimore) 1971; 50: 85-95.
199.
Jacobsen S. , Petersen J., Ullman S., Junker P., Voss A., Rasmussen JM, et al. A multicentre study of 513 Danish patients with systemic lupus erythematosus. I. Disease manifestations and analyses of clinical subsets. Clin Rheumatol1998; 17: 468-477.
200.
Bankier AA , Kiener HP, Wiesmayr MN, Fleischmann D., Kontrus M., Herold CJ, et al. Discrete lung involvement in systemic lupus erythematosus: CT assessment. Radiology1995; 196: 835-840.
201.
Sant SM, Doran M., Fenelon HM, and Breatnach ESPleuropulmonary abnormalities in patients with systemic lupus erythematosus: assessment with high resolution computed tomography, chest radiography and pulmonary function tests. Clin Exp Rheumatol1997; 15: 507-513.
202.
Matthay RA , Schwarz MI, Petty TL, Stanford RE, Gupta RC, Sahn SA et al. Pulmonary manifestations of systemic lupus erythematosus: review of twelve cases of acute lupus pneumonitis. Medicine ( Baltimore) 1975; 54: 397-409.
203.
Colby TVPulmonary pathology in patients with systemic autoimmune diseases . Clin Chest Med1998; 19: 587-612, vii.
204.
Min JK , Hong YS, Park SH, Park JH, Lee SH, Lee YS, et al. Bronchiolitis obliterans organizing pneumonia as an initial manifestation in patients with systemic lupus erythematosus . J Rheumatol1997; 24: 2254-2257.
205.
Filipek MS , Thompson ME, Wang P., Gosselin MV, and L. Primack, S Lymphocytic interstitial pneumonitis in a patient with systemic lupus erythematosus: radiographic and high-resolution CT findings. J Thorac Imaging2004; 19: 200-203.
206.
Cheema GS and Quismorio FPJ.Interstitial lung disease in systemic lupus erythematosus. Curr Opin Pulm Med2000; 6: 424-429.
207.
Weinrib L. , Sharma OP, and Quismorio FPJ.A longterm study of interstitial lung disease in systemic lupus erythematosus. Semin Arthritis Rheum1990; 20: 48-56.
208.
Swigris JJ , Olson AL, Fischer A., Lynch DA, Cosgrove GP, Frankel SK, et al. Mycophenolate mofetil is safe, well tolerated, and preserves lung function in patients with connective tissue disease-related interstitial lung disease. Chest2006; 130: 30-36.
209.
Carette S. , Macher AM, Nussbaum A., and Plotz PHSevere, acute pulmonary disease in patients with systemic lupus erythematosus: ten years of experience at the National Institutes of Health. Semin Arthritis Rheum1984; 14: 52-59.
210.
Ioachimescu OC and Stoller JKDiffuse alveolar hemorrhage: diagnosing it and finding the cause. Cleve Clin J Med2008 ; 75: 258, 260, 264-265 passim.
211.
Memet B. and Ginzler EMPulmonary manifestations of systemic lupus erythematosus. Semin Respir Crit Care Med2007; 28: 441-450.
212.
Zamora MR, Warner ML, Tuder R., and Schwarz MIDiffuse alveolar hemorrhage and systemic lupus erythematosus. Clinical presentation, histology, survival, and outcome. Medicine ( Baltimore) 1997; 76: 192-202.
213.
Barile LA, Jara LJ, Medina-Rodriguez F., García-Figueroa JL, and Miranda-Limόn JMPulmonary hemorrhage in systemic lupus erythematosus . Lupus1997; 6: 445-448.
214.
Schwab EP, Schumacher HRJ, Freundlich B., and Callegari PEPulmonary alveolar hemorrhage in systemic lupus erythematosusSemin Arthritis Rheum1993; 23: 8-15.
215.
Badsha H., Teh CL, Kong KO, Lian TY, and Chng HHPulmonary hemorrhage in systemic lupus erythematosus. Semin Arthritis Rheum2004; 33: 414-421.
216.
Santos-Ocampo AS, Mandell BF, and Fessler BJAlveolar hemorrhage in systemic lupus erythematosus: presentation and management. Chest2000; 118: 1083-1090.
217.
Chetan G., Mahadevan S., Sulanthung K., and Narayanan P.Intravenous immunoglobulin therapy of lupus pneumonitis. Indian J Pediatr2007; 74: 1032-1033.
Erkan D., Cervera R., and Asherson RACatastrophic antiphospholipid syndrome: where do we stand?Arthritis Rheum2003; 48: 3320-3327.
220.
Cervera R., Piette J., Font J., Khamashta MA, Shoenfeld Y., Camps MT, et al. Antiphospholipid syndrome: clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients. Arthritis Rheum2002; 46: 1019-1027.
221.
Asherson RAThe catastrophic antiphospholipid syndrome, 1998. A review of the clinical features, possible pathogenesis and treatment . Lupus1998; 7 Suppl2: S55-S62.
222.
Asherson RA , Cervera R., Piette JC, Shoenfeld Y., Espinosa G., Petri MA, et al. Catastrophic antiphospholipid syndrome: clues to the pathogenesis from a series of 80 patients . Medicine (Baltimore) 2001; 80: 355-377.
223.
Sinico RA, Di Toma L., Sabadini E., Renoldi P., and Li Vecchi M.Catastrophic antiphospholipid syndrome: report of 4 cases. J Nephrol2007; 20: 739-744.
224.
Simonson JS , Schiller NB, Petri M., and Hellmann DBPulmonary hypertension in systemic lupus erythematosus . J Rheumatol1989; 16: 918-925.
225.
Quadrelli SA, Alvarez C., Arce SC, Paz L., Sarano J., Sobrino EM, et al. Pulmonary involvement of systemic lupus erythematosus: analysis of 90 necropsies. Lupus2009; 18: 1053-1060.
226.
Shen JY, Chen SL, Wu YX, Tao RQ, Gu YY, Bao CD, et al. Pulmonary hypertension in systemic lupus erythematosus. Rheumatol Int1999; 18: 147-151.
227.
Winslow TM , Ossipov MA, Fazio GP, Simonson JS, Redberg RF, and Schiller NBFive-year follow-up study of the prevalence and progression of pulmonary hypertension in systemic lupus erythematosus . Am Heart J1995; 129: 510-515.
228.
Prabu A., Patel K., Yee C., Nightingale P., Situnayake RD, Thickett DR, et al. Prevalence and risk factors for pulmonary arterial hypertension in patients with lupusRheumatology ( Oxford) 2009; 48: 1506-1511.
229.
Gonzalez-Lopez L., Cardona-Muñoz EG, Celis A., García-de la Torre I., Orozco-arocio G., Salazar-Paramo M., et al. Therapy with intermittent pulse cyclophosphamide for pulmonary hypertension associated with systemic lupus erythematosus. Lupus2004; 13: 105-112.
230.
Jais X., Launay D., Yaici A., Le Pavec J., Tchérakian C., Sitbon O., et al. Immunosuppressive therapy in
231.
lupus- and mixed connective tissue disease-associated pulmonary arterial hypertension: a retrospective analysis of twenty-three cases. Arthritis Rheum2008; 58: 521-531.
232.
Condliffe R., Kiely DG, Peacock AJ, Corris PA, Gibbs JSR, Vrapi F., et al. Connective tissue disease-associated pulmonary arterial hypertension in the modern treatment era. Am J Respir Crit Care Med2009; 179: 151-157.
233.
Hennigan S. , Channick RN, and Silverman GJRituximab treatment of pulmonary arterial hypertension associated with systemic lupus erythematosus: a case report. Lupus2008 ; 17: 754-756.
234.
Kinder BW, Collard HR, Koth L., Daikh DI, Wolters PJ, Elicker B., et al. Idiopathic nonspecific interstitial pneumonia: lung manifestation of undifferentiated connective tissue disease?Am J Respir Crit Care Med2007; 176: 691-697.
235.
Bodolay E. , Szekanecz Z., Dévényi K., Galuska L., Csípo I., Vègh J., et al. Evaluation of interstitial lung disease in mixed connective tissue disease (MCTD). Rheumatology (Oxford) 2005 ; 44: 656-661.
236.
Prakash UBRespiratory complications in mixed connective tissue disease. Clin Chest Med1998; 19: 733-46, ix.
237.
Sullivan WD , Hurst DJ, Harmon CE, Esther JH, Agia GA, Maltby JD, et al. A prospective evaluation emphasizing pulmonary involvement in patients with mixed connective tissue disease. Medicine (Baltimore ) 1984; 63: 92-107.
238.
Raghu G., Brown KK, Costabel U., Cottin V., du Bois RM, Lasky JA, et al. Treatment of idiopathic pulmonary fibrosis with etanercept: an exploratory, placebo-controlled trial. Am J Respir Crit Care Med2008; 178: 948-955.
239.
King TEJ , Behr J., Brown KK, du Bois RM, Lancaster L., de Andrade JA, et al. BUILD-1: a randomized placebo-controlled trial of bosentan in idiopathic pulmonary fibrosisAm J Respir Crit Care Med2008; 177: 75-81.
240.
King TEJ , Albera C., Bradford WZ, Costabel U., Hormel P., Lancaster L., et al. Effect of interferon gamma-1b on survival in patients with idiopathic pulmonary fibrosis (INSPIRE): a multicentre, randomised, placebo-controlled trial. Lancet2009; 374: 222-228.
241.
Demedts M. , Behr J., Buhl R., Costabel U., Dekhuijzen R., Jansen HM, et al. High-dose acetylcysteine in idiopathic pulmonary fibrosis. N Engl J Med353, 2229-2242.
242.
Taniguchi H. , Ebina M., Kondoh Y., Ogura T., Azuma A., Suga M., et al. Pirfenidone in idiopathic pulmonary fibrosis. Eur Respir J2010;35: 821-829.
243.
Baughman RP and Lower EEUse of intermittent, intravenous cyclophosphamide for idiopathic pulmonary fibrosis. Chest1992 ; 102: 1090-1094.
244.
Dayton CS, Schwartz DA, Helmers RA, Pueringer RJ, Gilbert SR, Merchant RK et al. Outcome of subjects with idiopathic pulmonary fibrosis who fail corticosteroid therapy. Implications for further studies. Chest1993; 103: 69-73.
245.
Daoussis D. , Liossis SC, Tsamandas AC, Kalogeropoulou C., Kazantzi A., Sirinian C., et al. Experience with rituximab in scleroderma: results from a 1-year, proof-of-principle study . Rheumatology ( Oxford) 2010;49: 271-280.
