Vasculitis clinical presentation

Vasculitis usually is seen in patients with long-standing rheumatoid arthritis. Vasculitis may result in a wide variety of clinical presentations. Invasion of blood vessel walls by inflammatory cells results in an obliteration of the vessel, producing infarction of tissue distal to the area of involvement. Most commonly, small-vessel vasculitis produces infarcts near the ends of the fingers or toes, especially around the nail beds. These infarcts are usually of little consequence. 

INTRAVASCULAR LYMPHOMA The CNS is One of the sites of predilection for intravascular lymphoma, a large B-cell lymphoma. The neoplastic cells fill the blood vessel lumina. The clinical presentation mimics vasculitis. Neoplastic cells are positive for CD20. 

Henoch-Schoenlein purpura (HSP) is a systemic vasculitis affecting the skin, joints, gut, and kidneys. Nephritis in HSP (HSPN) and IgAN seem to be related diseases. Both have mesangial deposition of IgA as the dominating feature in renal biopsy specimens and thus can be discussed in combination. Clinical presentation is typical renal manifestation is present in up to 80% of patients mostly with isolated haematuria. Presentation with acute nephritic syndrome and renal failure or with nephrotic syndrome is rare, but indicates a progressive course of the disease. 

Cutaneous vasculitis is often a feature of such cases, although severe systemic manifestations often also occur. Two patients with propylthiouracil hypersensitivity presented with skin manifestations but also had renal, rheu-matological, and hematological features (66). A review of the literature showed that the symptoms and signs in patients with ANCA-associated thionamide-induced vasculitis are diverse. Acral purpuric skin lesions are typically seen recognition of these classical clinical features may allow early diagnosis and limit associated morbidity and the requirement for other therapies, particularly immunosuppression. Several other reports have described... 

Clinical features of ARN must include (1) focal well-demarcated areas of retinal necrosis located in the retinal periphery, (2) rapid circumferential progression of necrosis, (3) evidence of occlusive vasculitis, and (4) moderate to severe anterior chamber and vitreal inflammation. Mild presentations may manifest low-grade anterior chamber inflammation with or without blurred vision, whereas severe cases may include episcleritis, scleritis, and pain on eye movement. Early clinical findings include anterior and posterior uveitis, keratic precipitates, and presence of vitreous cells. Within several days to weeks, the patient develops dramatic progressive retinal whitening in multifocal and confluent patches, vasculitis of both retinal arteries and veins, and possible optic nerve head... 

The liver shows vasculitis with necrotizing epithelioid cell granulomas. Non-specific reactive hepatitis is also present. Therapy is with cyclophosphamide (1-2 mg/kg BW/day). We have given an extensive description of this clinical picture based on 132 cases in the literature as well as on our own experience. (84)... 

Another clinical classification of four cases of PTU-induced ANCA-associated vasculitis showed skin involvement to be evident in three of the four patients and renal involvement in two of the four patients. All were positive for cytoplasmic ANCA but only one presented with perinuclear ANCA [48 ]. Another case study 35-year-old woman presented with ANCA-associated vasculitis after switching from PTU to MMI. 

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