The objective of this article is to assess disease activity patterns and their relationship to damage, death and growth failure in a cohort of juvenile lupus.
Methods
Chronic active, relapsing-remitting and long quiescent activity patterns were retrospectively classified according to longitudinal scores of both the Modified SLEDAI-2K and ECLAM. The Pediatric SLICC/ACR Damage Index (Ped-SDI) was scored at the last visit in patients followed more than six months. Survival analysis was performed considering death, damage and growth failure, and stratified according to disease activity patterns. Cox model analysis identified predictors for damage and growth failure among onset clinical variables.
Results
Thirty-seven patients with 11 years mean age at diagnosis and 3.2 years mean follow-up were studied. According to the Modified SLEDAI-2K, activity pattern was 67.5% relapsing-remitting, 29.8% chronic active and 2.7% long quiescent and by ECLAM, 45.9%, 48.7% and 5.4%, respectively. The five-year survival was 90%. Damage accrued in 62.5% and growth failure in 31.3%. Chronic active cases progressed to damage earlier than relapsing-remitting (log-rank test, p < 0.05). Damage was associated with disease duration (p < 0.0001), thrombocytopenia (p < 0.05) and alopecia (p < 0.004). Growth failure was associated with disease duration (p < 0.007) and renal failure (p < 0.007).
Conclusion
Damage was observed in nearly two-thirds of patients, and occurred earlier in the chronic active pattern. Disease duration, thrombocytopenia and alopecia at onset predicted damage.
SilvermanEEddyASystemic lupus erythematosus. In: CassidyJTPettyRELaxerRMLindsleyCB (eds). Textbook of pediatric rheumatology, 6th ed. Philadelphia: Elsevier Saunders, 2011, pp. 315–343.
2.
BarrSGZonana-NacachAMagderLSPetriM. Patterns of disease activity in systemic lupus erythematosus. Arthritis Rheum1999; 12: 2682–2688.
3.
ZenMBassiNNalottoL. Disease activity patterns in a monocentric cohort of SLE patients: A seven-year follow-up study. Clin Exp Rheumatol2012; 30: 856–863.
4.
OttenMHCransbergKvan RossumMA. Disease activity patterns in juvenile systemic lupus erythematosus and its relation to early aggressive treatment. Lupus2010; 19: 1550–1556.
5.
BombardierCGladmanDDUrowitzMBCaronDChangCH. Committee on Prognosis Studies in SLE. Derivation of the SLEDAI. A disease activity index for lupus patients. Arthritis Rheum1992; 35: 630–640.
UribeAGViláLMMcGwinGJrSanchezMLReveilleJDAlarcόnGS. The Systemic Lupus Activity Measure-revised, the Mexican Systemic Lupus Erythematosus Disease Activity Index (SLEDAI), and a modified SLEDAI-2K are adequate instruments to measure disease activity in systemic lupus erythematosus. J Rheumatol2004; 31: 1934–1940.
8.
BrunnerHIFeldmanBMBombardierCSilvermanED. Sensitivity of the Systemic Lupus Erythematosus Disease Activity Index, British Isles Lupus Assessment Group Index, and Systemic Lupus Activity Measure in the evaluation of clinical change in childhood-onset systemic lupus erythematosus. Arthritis Rheum1999; 42: 1354–1360.
9.
GladmanDGinzlerEGoldsmithC. The development and initial validation of the SLICC/ACR damage index for systemic lupus erythematosus. Arthritis Rheum1996; 39: 363–369.
10.
Gutiérrez-SuárezRRupertoNGastaldiR. A proposal for a pediatric version of the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index based on the analysis of 1,015 patients with juvenile-onset systemic lupus erythematosus. Arthritis Rheum2006; 54: 2989–2996.
11.
NossentJKissERozmanB. Disease activity and damage accrual during the early disease course in a multinational inception cohort of patients with systemic lupus erythematosus. Lupus2010; 19: 949–956.
12.
LaustrupHVossAGreenAJunkerP. SLE disease patterns in a Danish population-based lupus cohort: An 8-year prospective study. Lupus2010; 19: 239–246.
13.
HochbergMC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum1997; 40: 1725–1725.
14.
VitaliCBencivelliWIsenbergDA. Disease activity in systemic lupus erythematosus: Report of the Consensus Study Group of the European Workshop for Rheumatology Research. II. Identification of the variables indicative of disease activity and their use in the development of an activity score. The European Consensus Study Group for Disease Activity in SLE. Clin Exp Rheumatol1992; 10: 541–547.
15.
BencivelliWVitaliCIsenbergDA. Disease activity in systemic lupus erythematosus: Report of the Consensus Study Group of the European Workshop for Rheumatology Research. III. Development of a computerised clinical chart and its application to the comparison of different indices of disease activity. The European Consensus Study Group for Disease Activity in SLE. Clin Exp Rheumatol1992; 10: 549–554.
16.
BrunnerHISilvermanEDBombardierCFeldmanBM. European Consensus Lupus Activity Measurement is sensitive to change in disease activity in childhood-onset systemic lupus erythematosus. Arthritis Rheum2003; 49: 335–341.
17.
FitzGeraldJDGrossmanJM. Validity and reliability of retrospective assessment of disease activity and flare in observational cohorts of lupus patients. Lupus1999; 8: 638–644.
18.
MoscaMBencivelliWVitaliC. The validity of the ECLAM index for the retrospective evaluation of disease activity in systemic lupus erythematosus. Lupus2000; 9: 445–450.
19.
KuczmarskiRJOgdenCLGrummer-StrawnLM. CDC growth charts: United States. Adv Data2000; 314: 1–27.
20.
LandisJRKochGG. The measurement of observer agreement for categorical data. Biometrics1977; 33: 159–174.
21.
SteimanAJUrowitzMBIbañezDPapnejaAGladmanDD. Prolonged clinical remission in patients with systemic lupus erythematosus. J Rheumatol2014; 41: 1808–1816.
22.
Gómez-PuertaJAPons-EstelBACerveraR. Prolonged remission in SLE revisited: An old wine in a new bottle. J Rheumatol2014; 41: 1728–1731.
WatsonLLeoneVPilkingtonC. Disease activity, severity, and damage in the UK Juvenile-Onset Systemic Lupus Erythematosus Cohort. Arthritis Rheum2012; 64: 2356–2365.
25.
DungNTLoanHTNielsenSZakMPetersenFK. Juvenile systemic lupus erythematosus onset patterns in Vietnamese children: A descriptive study of 45 children. Pediatr Rheumatol Online J2012; 10: 38–38.
26.
RavelliADuarte-SalazarCBurattiS. Assessment of damage in juvenile-onset systemic lupus erythematosus: A multicenter cohort study. Arthritis Rheum2003; 49: 501–507.
27.
BandeiraMBurattiSBartoliM. Relationship between damage accrual, disease flares and cumulative drug therapies in juvenile-onset systemic lupus erythematosus. Lupus2006; 15: 515–520.
28.
RyggMPistorioARavelliA. A longitudinal PRINTO study on growth and puberty in juvenile systemic lupus erythematosus. Ann Rheum Dis2012; 71: 511–517.
29.
Becker-MerokANossentHC. Damage accumulation in systemic lupus erythematosus and its relation to disease activity and mortality. J Rheumatol2006; 33: 1570–1577.
30.
BrunnerHISilvermanEDToTBombardierCFeldmanBM. Risk factors for damage in childhood-onset systemic lupus erythematosus: Cumulative disease activity and medication use predict disease damage. Arthritis Rheum2002; 46: 436–444.
31.
ZiakasPDDafniUGGiannouliSTzioufasAGVoulgarelisM. Thrombocytopaenia in lupus as a marker of adverse outcome—seeking Ariadne’s thread. Rheumatology (Oxford)2006; 45: 1261–1265.
32.
FernándezMAlarcónGSApteM. Systemic lupus erythematosus in a multiethnic US cohort: XLIII. The significance of thrombocytopenia as a prognostic factor. Arthritis Rheum2007; 56: 614–521.
33.
Zonana-NacachAYañezPJiménez-BalderasFJCarmargo-CoronelA. Disease activity, damage and survival in Mexican patients with acute severe systemic lupus erythematosus. Lupus2007; 16: 997–1000.
34.
ZhaoHLiSYangR. Thrombocytopenia in patients with systemic lupus erythematosus: Significant in the clinical implication and prognosis. Platelets2010; 21: 380–385.
35.
PetriMOrbaiAMAlarcónGS. Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum2012; 64: 2677–2686.
36.
TanEMCohenASFriesJF. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum1982; 25: 1271–1277.
37.
LillebyVFlatøBFørreO. Disease duration, hypertension and medication requirements are associated with organ damage in childhood-onset systemic lupus erythematosus. Clin Exp Rheumatol2005; 23: 261–269.
