A clinical case of recurrent episcleritis as the initial manifestation of granulomatosis with polyangiitis

Abstract

Granulomatosis with polyangiitis (GPA) is a type of small-sized blood vessel vasculitis that predominantly affects the upper airways, lungs and kidneys and associates with the presence of anti-neutrophil cytoplasmic antibodies (ANCA). Nevertheless, any organ of the body can be affected by GPA, including the eye. Occasionally, ocular involvement can be the initial manifestation, thus representing an essential clue for the physician in the early diagnosis of the disease. We present the case of a 53-year-old woman in whom recurrent episcleritis was the first sign of a multisystem disease. All further investigations led to the final diagnosis of GPA. The remission induction therapy chosen by the rheumatologist consisted of intravenous cyclophosphamide (CP) and methylprednisolone pulse-therapy, followed by oral glucocorticoids (GC). Based on the favorable clinical and paraclinical evolution, induction therapy was replaced by remission maintenance therapy. Azathioprine (AZA) was initiated and oral GC were continued, with dose tapering. Complete remission of episcleritis was observed. Abbreviations: GPA = granulomatosis with polyangiitis, EGPA = eosinophilic granulomatosis with polyangiitis, MPA = Microscopic polyangiitis, ANCA = Anti-neutrophil cytoplasmic antibodies, c-ANCA = ANCA to proteinase-3, p-ANCA = ANCA to myeloperoxidase, ELISAs = antigen-specific enzyme-linked immunosorbent assays, ENT = ear, nose, throat, CP = cyclophosphamide, NSAIDs = nonsteroidal anti-inflammatory drugs, AZA = azathioprine, GC = glucocorticoids.

Keywords: cyclophosphamide; episcleritis; glucocorticoids; granulomatosis with polyangiitis; kidney biopsy; nasal biopsy.

Fig. 1

Left eye episcleritis; different stages of evolution under treatment

Fig. 2

Renal biopsy. A. Renal parenchyma with glomerular mesangial cell proliferation (black arrow). B. Renal parenchyma with global glomerulosclerosis (black arrow)

Fig. 3

Nasal mucosa. A. Inflammatory cell infiltrate (lymphocytes, neutrophils, histiocytes) of the lamina propria (10x). B. Subepithelial infiltrate of neutrophils and red blood cells (black arrow) – suggestive of vasculitis (40x)

Fig. 4

c-ANCA titer evolution during patient follow-up