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Cardiac and central nervous system vasculitis in a child with dermatomyositis.

Cerebral vasculitis and clinically important myocardial inflammation are rare in juvenile dermatomyositis. We report a previously healthy 6-year-old girl with dermatomyositis who died after a fulminating clinical deterioration. Postmortem examination of the heart revealed characteristic endothelial tubuloreticular aggregates and evidence of capillary necrosis and secondary thrombosis, associated with extensive hemorrhagic myocardial necrosis. Endothelial necrosis was also evident in the cerebrocortical capillaries.

Autopsy↗

Systemic vasculitis in a patient with small-cell neuroendocrine bronchial cancer.

A 75-yr-old male hospitalized for vascular purpura with joint pain had a medical history of polymyalgia rheumatica. A generalized oedematous syndrome occurred and the patient also presented with haemoptysis and complained of transient paraesthesia of the hands and feet. Renal biopsy showed lesions of focal segmental proliferative glomerulonephritis associated with a few cellular crescents. Lung biopsy showed small-cell neuroendocrine carcinoma. After the first course of chemotherapy signs of vasculitis disappeared. Small-cell neuroendocrine carcinomas, which represent 25% of all lung cancers, have numerous paraneoplastic (especially neurological) extrapulmonary manifestations. Disseminated vasculitis has never been described with this type of cancer, whereas nonsmall-cell carcinomas are associated essentially with cutaneous vasculitis or purpura rheumatica. In the case reported here, anticancer chemotherapy allowed vasculitic manifestations to be treated.

Aged↗

Plasmapheresis in antineutrophil cytoplasmic antibody-associated systemic vasculitis.

Small vessel vasculitis syndromes associated with antineutrophil cytoplasmic antibodies frequently cause a necrotizing and crescentic glomerulonephritis with the potential to progress rapidly to permanent renal failure. These conditions are conventionally treated with immunosuppressive drugs, but the possibility that humoral factors are important in their pathogenesis has led to the evaluation of plasmapheresis as an adjunctive therapy. Both controlled and uncontrolled studies have suggested that the routine addition of plasmapheresis is unnecessary. However, when renal function is impaired to the point that dialysis is required, the addition of plasma exchange increases the chance of renal recovery. The superiority of this approach over pulses of methylprednisolone remains to be confirmed.

Antibodies, Antineutrophil Cytoplasmic↗

Skin fibrinolytic activity in cutaneous and systemic vasculitis.

Study of involved and uninvolved skin from patients with necrotizing vasculitis revealed diminished tissue fibrinolytic activity deposition of immunoreactants in involved skin. In these patients, the depletion of tissue fibrinolytic activity is probably the result of vessel injury secondary to the local deposit of immunoreactants. In addition, there was diminished tissue fibrinolytic activity in uninvolved skin from patients with and without clinical skin involvement, unassociated with the deposition of immunoreactants. The precise mechanism for diminished tissue lytic activity in these latter patients is not known, but it may be associated with generalized activation of the coagulation and fibrinolytic mechanisms that result in local depletion of tissue fibrinolytic activity. These local changes may aggravate the clinical course of the disease as well as inhibit the healing of the lesions.

Adult↗