A case report of the orbit, ocular association and the lung in granulomatosis with polyangiitis: A diagnostic challenge

Abstract

Granulomatosis with polyangiitis (GPA) is a systemic form of vasculitis that involves small to medium sized vessels and is associated with high morbidity and mortality. GPA presents a continuous and difficult clinical diagnostic concern, due to the rarity of the disease and the diversity of the manifestations. This case report discusses the unusual symptoms presented by a particular patient, discusses these manifestations and explains how the final diagnosis was identified as GPA. A 40-year old Chinese woman was initially referred to the present institution for a progressive worsening pain, redness and gradual decrease in visual acuity in the eyes over the past 7-year period. Previous therapeutic interventions included noncompliant topical and intravenous dexamethasone for 6 years. A pre-operative examination conducted in a differing hospital to search for the presence of an orbital mass, resulted in the identification of an asymptomatic space-occupying lesion in the right middle lung, which was surgically removed in March 2015. A total of four weeks later, surgery was then applied to remove a left orbital mass, in the same hospital. A total of three months later, the patient was diagnosed with peripheral ulcerative keratitis associated with GPA, at the present institution. The corneal lesions were then treated bilaterally with cryotherapy and oral prednisone and cyclophosphamide were administered. Following surgery, the condition of the eyes appeared to be stable. A total of seven months later, the redness and pain of the right eye recurred, followed by a deep lamellar keratoplasty for the treatment of necrotizing scleritis. The condition of the two eyes was subsequently observed to be stable during the nine month follow-up. The present case study reviews various points to consider in a rare, complicated and potentially blinding case of GPA. GPA must therefore be considered in the differential diagnosis of further inflammatory conditions and tumors. Early diagnosis and an appropriate interdisciplinary approach to management, are required to decrease recurrence and morbidity in patients with GPA-mediated inflammatory ocular disease.

Keywords: granulomatosis with polyangiitis; lung lesions; orbital mass; peripheral ulcerative keratitis.

Figure 1.

Montage of radiographic illustrations for left orbital mass and lung lesions. (A) The chest CT Scan showed a size of 46×49 mm quasi-circular shaped space-occupying lesion with unevenly thickened walls and spiculated boarders in lateral segment of the middle lobe of right lung. (B) The orbital CT showed a poorly defined complex mass involving the left rectus medialis muscle and rectus inferior muscle, located in the inner lower quadrant of the left orbital outside the muscle cone.

Figure 2.

Montage of histopathology illustrations for left orbital mass and cavitary lesions from lung. (A) Histopathology of cavitary lesions from right middle lung showing granulomatous inflammation, necrotizing vasculitis with aggregate of inflammatory cells (H&E staining, ×200). (B) Histopathology of lesions from left orbital mass illustrated hyperplasia of fibrous tissue infiltrated by dense inflammatory cells, granuloma formation and focal necrosis (H&E staining, ×200).

Figure 3.

Montage of clinical illustrations. (A) Slit lamp examination showed peripheral ulcerative keratitis, posterior synechia of the iris and necrotizing scleritis of the right eye. (B) Slit lamp examination showed worsened healed peripheral ulcerative keratitis after deep lamellar kemtoplasty.