Abstract
Purpose
To describe a case series of scleritis associated with IgA vasculitis (IgAV) at a tertiary referral center.
Observations
Three men with scleritis associated with IgAV were identified: one with anterior scleritis alone, one with anterior scleritis and peripheral ulcerative keratitis (sclerokeratitis), and one with anterior and posterior scleritis. Visual acuity was preserved except from the patient who developed posterior scleritis. Ocular pain was the main symptom at presentation. All patients had a previous history of palpable purpura, but only one was aware of his underlying IgAV. Laboratory results revealed microhematuria and proteinuria with normal urinary β 2 microglobulin levels and negative serum ANCAs. Skin or kidney biopsy demonstrated leukocytoclastic vasculitis or glomerulonephritis with dominant IgA immune deposits.
Conclusions and Importance
Although uncommon, IgAV should be included in the differential diagnosis of anterior scleritis alone or associated with peripheral ulcerative keratitis or posterior scleritis, even in systemically asymptomatic patients. Urinalysis should not be underestimated in assessment of scleritis to detect early stages of glomerular disease. Scleritis may be the first manifestation whose study may lead to the diagnosis of IgAV. Multidisciplinary approach is necessary to prevent irreversible organ damage such as renal failure.
1
Introduction
Scleritis is a sight-threatening ocular condition that can occur as an isolated phenomenon or associated with immune diseases or infections. Systemic autoimmune or vasculitis diseases can be found in 48% of cases. Immune-complex deposition in the vessel walls is suggested to play an important role in the pathogenesis of scleritis. , Immunoglobulin A vasculitis (IgAV) is an immune complex-mediated disease affecting small vessels (predominantly capillaries, venules, or arterioles) with IgA1-dominant immune deposits presenting with purpura, arthralgias, abdominal pain, and renal disease as the classic tetrad. A classification criteria for IgAV proposed in 2010 by the European League Against Rheumatism/Pediatric Rheumatology International Trials Organisation/Pediatric Rheumatology European Society (EULAR/PRINTO/PRES) considered the presence of purpura or petechiae, mainly in lower limbs, as mandatory criterion together with at least one of the other four criteria: arthritis/arthralgia, abdominal pain, glomerular involvement (proteinuria and/or hematuria), and IgA deposits in a tissue biopsy (skin or kidney) associated with leukocytoclastic vasculitis or proliferative glomerulonephritis. These criteria provide 100% sensitivity and 87% specificity for classifying patients with IgAV. Purpura is the most common manifestation; skin biopsy shows leukocytoclastic vasculitis, an inflammation of the small blood vessels, most prominently in the postcapillary venules. Gastrointestinal tract and renal involvement represent the main causes of morbidity and mortality; in a large study of 250 adults with IgAV, 11% of patients reached end-stage renal disease, 13% had several renal failure, and 14% had moderate renal failure. Treatment remains controversial because disease course ranges from spontaneous remission to end-stage renal disease and the initial presentation does not correlate with the outcome.
Ocular involvement in patients with IgAV is rare: anterior uveitis and episcleritis are the most common associations reported in literature. However, reports of scleritis and IgAV are very scarce.
Herein, we report a case series of three patients presenting with anterior scleritis alone or associated with peripheral ulcerative keratitis (PUK) or posterior scleritis, in which urinalysis was essential to reveal the undetected nephropathy. IgAV was finally confirmed by tissue biopsy and a multidisciplinary assessment was implemented to prevent further organ damage.
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Methods
The electronic health records were reviewed of patients with scleritis associated with IgAV seen at our tertiary referral center from 2015 to 2020. Collected data included age, gender, ophthalmologic examination, and ancillary tests. A comprehensive medical and ophthalmic history was elicited in each patient. Ophthalmologic examination included best-corrected visual acuity (BCVA, Snellen), intraocular pressure (IOP), slit-lamp anterior segment assessment, and dilated fundus examination. Anterior segment photographs and anterior and posterior segment swept-source optical coherence tomography (SS-OCT, Triton, Topcon) images of the altered sclera were analyzed at presentation and follow up. Anterior chamber activity was graded using SUN (Standardization of Uveitis Nomenclature) scale. Extensive workup was undertaken in all patients with complete blood counts, serum chemistry analysis, serum antineutrophil cytoplasmic antibodies (ANCAs), urinalysis, and β 2 microglobulin in urine. Additional testing was carried out when positive signs or symptoms were found in the review of systems to rule out infectious or autoimmune diseases. IgAV was confirmed based on clinical and histological findings. A multidisciplinary approach with autoimmune disease and nephrology departments was required to assess systemic manifestations.
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Findings
Three patients with anterior scleritis associated with IgAV were identified: one presenting with anterior scleritis alone, one combined with PUK (sclerokeratitis) and one combined with posterior scleritis. Median age was 45 years-old (range: 42–55) and all were male.
All patients had a past history of palpable purpura affecting the lower extremities. Final diagnosis of leukocytoclastic vasculitis was confirmed after skin biopsy in two cases. However, IgA deposits in direct immunofluorescence were demonstrated only in one case (Case 1). IgAV was the underlying disease in the three cases, as confirmed by kidney (two cases) or skin biopsy (one case).
At the time of the scleral inflammation, one patient was already on treatment with systemic immunosuppressive therapy (azathioprine, dapsone and prednisone) to control extra-renal IgAV manifestations (leukocytoclastic vasculitis and arthritis).
Interestingly, all cases developed systemic manifestations before ocular manifestations occurred: IgA nephritis, 30 years prior, and palpable purpura, three years prior (Case 1); leukocytoclastic vasculitis, two years prior (Case 2); leukocytoclastic vasculitis, arthritis, and abdominal pain, two years prior (Case 3).
All patients presented with ocular pain as the major symptom. Slit-lamp examination showed mild anterior chamber cells (SUN scale) only in the patient with sclerokeratitis (Case 2). BCVA was preserved, except from the patient who developed posterior scleritis (Case 3). Scleral thickness measured by SS-OCT was increased in the affected areas.
Regarding clinical course, all cases showed flares of alternating anterior scleritis despite oral nonsteroidal anti-inflammatory drugs (NSAIDs). Urinary β 2 microglobulin levels and serum ANCAs were normal in all cases.
Baseline ocular and renal characteristics are outlined in the Table. Individual cases are briefly described below:
3.1
Case 1
A 42-year-old man presented with ocular pain in his right eye (OD) at our emergency department. He was a former heavy smoker. Past medical history through shared electronic health record revealed a kidney biopsy during his childhood consistent with IgAV. In addition, he suffered from palpable purpuric lesions on his right lower extremity three years prior. Slit lamp examination showed diffuse anterior scleritis OD in the temporal inferior quadrant ( Fig. 1 A). BCVA and IOP were preserved. No anterior chamber cells were detected and funduscopy was normal. He was diagnosed with diffuse anterior scleritis OD and he was subsequently started on oral ibuprofen 600mg tid improving at one week ( Fig. 1 B) and resolving at one month ( Fig. 1 C). Further investigations revealed serum creatinine of 1,3 mg/dL (range: 0,3-1,3), microhematuria, and proteinuria (666 mg/d; range: <150). Accordingly, he was referred to nephrology department. A new kidney biopsy confirmed the suspected diagnosis ( Fig. 1 D and E) and he was started on an oral angiotensin receptor blocker (ARB) for nephroprotection. Anterior segment SS-OCT revealed scleral and episcleral thickening ( Fig. 1 F). During follow up, recurrent episodes (twice yearly) of alternating anterior scleritis were noted with good response to oral ibuprofen. He continued follow up with nephrology department.
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