A 3-year-old Hispanic girl with no prior history of systemic disease was sent for a consultation to our uveitis service for the further evaluation of her bilateral granulomatous uveitis. Her family history was remarkable for PsA in her father and uncle. The review of systems revealed that she had a 3-month history of a skin eruption and left knee arthralgia.
Upon a comprehensive examination, her best-corrected visual acuity (BCVA) was 20/60 in both eyes. The intraocular pressure was 13 mmHg in the right eye (OD) and 14 mmHg in the left eye (OS). A slit-lamp exam revealed bilateral band keratopathy, large mutton-fat keratic precipitates (KPs), multiple posterior synechiae (Figures 1(a)-1(d)), and 4+ anterior chamber cells. The fundus view was very poor for both eyes due to the presence of band keratopathy and limited pupillary dilation. A B-scan ultrasound was unremarkable, with no evidence of vitreous opacity.
The physical examination was remarkable for left knee edema and a right axillary nonpruritic eruption consisting of irregularly shaped erythematous papules and plaques with thin scale (Figure 2(a)). No dactylitis, enthesitis, or nail changes were identified. As the differential diagnosis of granulomatous iridocyclitis includes infectious and noninfectious etiologies, a diagnostic work-up was ordered, and the patient was started on hourly topical prednisolone acetate 1% and atropine sulfate 1%, the latter, twice a day.
The radiological images were remarkable only for left knee effusion. The girl's chest X-ray was normal, with no lymphadenopathy, opacities, or cavitations. Routine laboratory tests revealed an erythrocyte sedimentation rate of 80 mm/h and an unremarkable complete metabolic panel (CMP) (Na: 141 mEq/L, K: 3.7 mEq/L, Cl: 103 mEq/L, blood urea nitrogen: 9.00 mg/dL, and serum creatinine: 0.25 mg/dL) and urinalysis (negative for glucose, protein, red blood cells, white blood cells, and casts). Her immunology work-up was remarkable for a positive ANA test (1 : 1,280), with a homogenous pattern and negative anti-double-stranded DNA antibody, anti-Smith antibody, rheumatoid factor (<10 IU/mL), and cyclic citrullinated peptide IgG. Fluorescent treponemal antibody absorption (FTA-ABS), venereal disease research laboratory (VDRL), and tuberculosis interferon-gamma release assay (IGRA) tests were negative. Serological tests for the human leukocyte antigen B27 haplotype, HIV, hepatitis A IgM, hepatitis B core IgG, and the hepatitis C antibody were negative.
A punch biopsy of a skin lesion revealed psoriasiform acanthosis, thin supra-papillary plates, neutrophils in the stratum corneum, and superficial perivascular lymphocytic infiltrate (Figure 2(b)). Based on these results, the uveitis specialist concluded a diagnosis of bilateral granulomatous iridocyclitis associated with early-onset JPsA.
After establishing a tissue diagnosis of JPsA, oral prednisolone (1 mg/kg) was added to her therapy. Following 2 weeks of treatment, her uveitis was inactive, bilaterally. The BCVA improved to 20/40 in OD and remained stable at 20/60 in OS. At the 2-month follow-up visit, her uveitis remained inactive, and her BCVA improved to 20/30 in OD and 20/40 in OS. At this visit, a standardized prednisolone tapering protocol was begun, and she was started on subcutaneous methotrexate (0.2 mg/kg/week) as a corticosteroid-sparing therapy.