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[Hemosorption in systemic vasculitis].

The present work summarises the experience gained with the use of extracorporeal hemoperfusion in 45 patients suffering from rheumatic diseases. During hemoperfusion, there was a decrease in the initially activated LDH, CPK, AST, ALT and AP. The reduction of the content of phospholipids and cholesterol in plasma and red blood cells is assessed as an adverse effect of hemoperfusion, since the content of phospholipids and cholesterol was under normal before hemoperfusion. The changes indicated point to the necessity of administering antioxidants and exogenous phospholipids.

Cholesterol↗

Systemic vasculitis associated with eosinophilia and marked degranulation of tissue eosinophils.

A 9-year-old girl had fever, life-threatening interstitial pneumonia, a vesicular skin rash, parotid and submandibular swelling, and marked blood eosinophilia (WBC count 47,000/microL, 39% eosinophils). Results of lung and skin biopsies showed vasculitis with intense eosinophil infiltration. Immunofluorescence analyses of these biopsies, as well as analysis of tissue from the parotid, lip, and trachea, showed striking deposition of eosinophil granule major basic protein associated with areas of tissue damage. Treatment with glucocorticoids and hydroxyurea produced clinical improvement. The association between tissue damage and deposition of the cytotoxic major basic protein suggests that the eosinophil contributed to the pathophysiology of this disease. Recognition of the capacity of the eosinophil to produce tissue damage can be helpful in the selection of therapy.

Blood Proteins↗

Pulmonary capillaritis and hemorrhage in patients with systemic vasculitis.

Thirteen patients with prominent pulmonary signs and symptoms had pulmonary capillaritis and extensive hemorrhage demonstrated by open-lung biopsy or autopsy. Vasculitis was demonstrated in other organs before or after the lung biopsy or at autopsy. Although there were several suspected causes for the pulmonary capillaritis and different final clinicopathologic diagnoses, the histopathologic features in the lung were similar in all cases and distinctive enough to separate capillaritis from other causes of hemorrhagic lung. All patients were treated with prednisone or cyclophosphamide, or both. Six patients died of their vasculitis, five in respiratory failure and one in renal failure. None of the seven survivors had a clinical recrudescence of pulmonary hemorrhage. By extrapolation from these 13 cases, one may histopathologically recognize pulmonary capillaritis when it causes hemorrhagic lung in a patient without clinically evident extrapulmonary vasculitis. One can then proceed to investigate the patient and possibly determine the nature of the vasculitis.

Adolescent↗