The clinical pathology of Crimean-Congo hemorrhagic fever.

Observations were made of 15 fatal and 35 nonfatal Crimean-Congo hemorrhagic fever (CCHF) infections diagnosed from February 1981 to March 1987 in Kimberly and Sandringham, Republic of South Africa. Following an incubation period of 2-9 days after exposure to infection, patients had a sudden onset of disease with fever, nausea, severe headache, and myalgia. Petechial rash and hemorrhagic signs such as epistaxis, hematemesis, and melena supervened on days 3-6 of illness. Deaths occurred on days 5-14 of illness. Patients with fatal infections had thrombocytopenia and markedly elevated levels of serum aspartate and alanine aminotransaminases, gamma-glutamyltransferase, lactic dehydrogenase, creatine kinase, bilirubin, creatinine, and urea. Total protein, albumin, fibrinogen, and hemoglobin levels were depressed. Values for prothrombin ratio, activated partial thromboplastin time, thrombin time, and fibrin degradation products were grossly elevated, findings that indicate the occurrence of disseminated intravascular coagulopathy. Many of the clinical pathologic changes were evident at an early stage of the disease and had a highly predictive value for fatal outcome of infection. Changes were present but less marked in nonfatal infections.

Cardiac involvement in mixed connective tissue disease. A fatal case of scleroderma combined with systemic lupus erythematosus.

A 27-year-old black woman with cardiac failure, angina pectoris and Raynaud's syndrome is presented. Skin biopsy and barium studies established the diagnosis of scleroderma (progressive systemic sclerosis (PSS)). Systemic lupus erythematosus (SLE) was strongly suggested by the results of immunological studies and increasing severity of renal failure. Because of the possibility of a cardiomyopathy, cardiac catheterization, selective coronary angiography and right ventricular endomyocardial biopsy were carried out but failed to show any histological features of either SLE or PSS. The patient went into progressive renal failure despite immunosuppressive therapy and plasmapheresis and died; consent for autopsy was refused. A final diagnosis of mixed connective tissue disease (MCTD) was made. The salient features of cardiac involvement in SLE, PSS and MCTD are outlined.