Granulomatosis with polyangiitis (GPA) is a primary, necrotizing granulomatous vasculitis, involving small- to medium-sized arteries, that causes systemic disease. Almost any organ can be affected, but the most affected systems are the upper airways, lungs, kidneys, eyes and peripheral nerves. Migratory polyarthritis is reported in approximately 25% of patients with anti-neutrophil cytoplasmic antibody (ANCA) associated…
A 67–year-old white woman with primary Raynaud’s phenomenon presented following a week of progressively worsening shortness of breath, dry cough and generalized malaise. An avid tennis player, she first noticed dyspnea while playing, but a few days later grew short of breath even at rest. She went to an urgent care center, where a computed…
A 42-year-old man with a history of ulcerative colitis (UC), primary sclerosing cholangitis (PSC) and chronic sinusitis was referred to a rheumatologist to evaluate for a possible diagnosis of systemic vasculitis. This patient had developed new skin lesions, gingival hypertrophy and ulcerating tracheobronchitis, concerning for possible granulomatosis with polyangiitis (GPA). Since 1994, the patient had…
Recurrent cutaneous eosinophilic vasculitis (RCEV) is a rare autoimmune condition characterized histologically by necrotizing small vessel vasculitis of the skin and almost exclusive eosinophilic infiltration without any systemic involvement.1 Frequently, there is associated peripheral eosinophilia, and a prolonged course of glucocorticoids is required for treatment. To date, only a few RCEV cases have been reported….
Antiphospholipid syndrome (APS) is an autoimmune condition characterized by hypercoagulability often manifested as recurrent thrombosis or pregnancy complications, with persistently circulating antiphospholipid (aPL) antibodies or lupus anticoagulant. Catastrophic APS (CAPS), also known as Asherson syndrome, occurs in less than 1% of cases of APS and involves occlusive microangiopathy in at least three organ systems.1 Case…
A 66-year-old man presented to the hospital with left-sided visual loss, headache, hematuria, and symptoms of a chronic sinusitis.