Conjunctivitis as the important indicator of pediatric granulomatosis with polyangiitis

Introduction

Granulomatosis with polyangiitis (GPA) disease, known as Wegner granulomatous, is a rare multisystem autoimmune disease with unknown etiology, which is characterized by granulomatosis inflammation, tissue necrosis, and vasculitis in small and medium-sized vessels. ¹ The main clinical characteristics of this disease include the upper and/or lower respiratory tract and kidneys. Ear, nose, and throat manifestations with recurrent sinusitis and crusting rhinorrhea are usually severe as well. ² in this regard, diagnosis of GPA is performed based on the clinical assessment, serological tests for antineutrophil cytoplasmic antibody (ANCA), and histological analysis. ³

One of the organs that may be involved in this disease is eye with various manifestations. In fact, conjunctivitis is considered as a very rare manifestation that appeared at the first stage in GPA.^4–7 In this study, the unusual case in whom bilateral conjunctivitis was the important indicator of GPA in adolescence is reported.

Case presentation

A 14-year-old boy was referred from ophthalmology clinic to the pediatric rheumatology ward of Imam Hossein Children’s Hospital, Isfahan University of Medical Sciences only due to drug-resistant conjunctivitis. On ophthalmic examination, anterior and posterior chambers and funduscopy of his both eyes were found to be completely normal. Moreover, visual acuity was 9/10 bilaterally. Of note, he was treated with prednisolone eye drop due to redness of both eyes, tearing, and photophobia for a 3-month period. As well, he was under intermittent therapy with montelukast, nasal steroid spray, and oral cetirizine for 2 years due to having constant rhinorrhea and nighttime coughing, so he was diagnosed with allergic rhinitis. Physical examination of the patient revealed bilateral nonpurulent conjunctivitis (Figure 1).

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Figure 1.

(a) Bilateral nonpurulent conjunctivitis at the first admission and (b) after treatment.

By outpatient testing, complete blood count (CBC) showed leukocytosis, thrombocytosis, and anemia, and erythrocyte sedimentation rate (ESR) was obtained as 115 mm/h. Based on these laboratory findings, the patient was admitted to rheumatology ward. In addition, urinalysis showed 3+ proteinuria and 2+ hematuria, and protein in 24-hour urine was 1450 mg/24 h. Thereafter, due to a history of chronic sinusitis, paranasal sinus computed tomography (CT) scan was done, which revealed maxillary and bilateral frontal sinusitis. Besides, spiral chest CT scan showed multiple pulmonary nodules in both lungs (Figure 2).

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Figure 2.

Multiple pulmonary nodules in both lungs.

Serum autoantibody levels were measured (antinuclear antibody), antiphospholipid antibodies and anti-DNA antibody (double-stranded DNA) were negative, but serum antineutrophil cytoplasmic antibody(C-ANCA) level was calculated as 450 IU/L, which was very high (NL <25 IU/L). Considering renal involvement and a high level of C-ANCA, renal biopsy was done. Its result confirmed ANCA-associated focal sclerosing glomerulonephritis with pauci-immune vasculitis.The patient was diagnosed with GPA in terms of the EULAR/PRINTO/PRES criteria and then methylprednisolone pulse 30 mg/kg daily (max: 1 g) and cyclophosphamide intravenous (IV) infusion were started for him, followed by oral prednisolone and cyclophosphamide IV infusion every 2 weeks for a 6-week duration after being discharged and then continued every 3 weeks. After 3 months, the conjunctivitis resolved and ocular examination through slit lamp and funduscopy was found to be normal (Figure 1).

Moreover, CBC was normal, serum C-ANCA level was 75 IU/L, ESR was 45 mm/h, and protein in 24-h urine was 350 mg. Thereafter, the treatment continued based on the EULAR/PRINTO/PRES recommendation.

Discussion

GPA is an ANCA-associated autoimmune disease, which involves small to medium-sized arteries and veins, which is characterized by necrotizing granulomatous inflammation. The common clinical characteristics of this disease include sinusitis, lung nodules, rhinorrhea, ear, and ocular manifestations. ²

Diagnosis of GPA is performed based on the laboratory and clinical findings. Laboratory tests play an important role in the diagnosis of GPA. In addition, it is noteworthy that serologic indicators of the generalized inflammation such as ESR and C-reactive protein (CRP) commonly increase in GPA. However, C3 and C4 complement levels may be reduced during this disease.^2,3

One of the organs that may be involved during the course of this disease is eye with various manifestations, including episcleritis, proptosis, scleritis, uveitis, conjunctivitis, and corneal ulceration.^4–6 A previous study has shown that the most ocular involvement at the time of diagnosis is scleritis, followed by proptosis and uveitis. However, at the time of diagnosis, no manifestation of conjunctivitis was found. ⁴ In fact, conjunctivitis is a very rare manifestation in GPA appearing at the first stage.^4,7 In the present study, we attempted to report the unusual case in which bilateral conjunctivitis was the first sign in adolescence.

The patient reported in this case study presented with drug-resistant conjunctivitis, which was diagnosed by the redness of both eyes, tearing, and photophobia for 3 months.

Conjunctivitis can be inflammation or infection of the translucent mucous membrane covering the anterior part of the sclera and inside the eyelids. Conjunctiva in GPA may be ulcerative, necrotic, or cicatricial. The common manifestations of conjunctivitis are irritation, itching, foreign body sensation, and watering or discharge.^8–11 Although the prevalence rate of ophthalmic manifestation in GPA is about 58%, conjunctivitis is known as a very rare first manifestation.^4,12,13

As the ocular manifestation of GPA is one of the most important signs of this disease, the collaboration between different specialists such as ophthalmologist and rheumatologist seems very essential in this regard.

Conclusion

Although ocular involvement sometimes occurs in GPA, this is the first report showing that conjunctivitis may be an important indicator of GPA in adolescence.