Cardiovascular involvement in systemic lupus erythematosus: an autopsy study of 27 patients in India.

BACKGROUND: Although cardiovascular disease (CVD) is recognized as a leading cause of death in patients with systemic lupus erythematosus (SLE) in western countries, there is hardly any data regarding Indian subjects with SLE. AIMS: To determine the incidence of cardiac abnormalities and vascular lesions at autopsy and to assess their contribution to the mortality in patients with SLE. SETTINGS AND DESIGN: Retrospective retrieval of reports of autopsies performed on 35 patients with SLE over a 11 year period and analysis of 27 cases with cardiac and/or vascular lesions. MATERIALS AND METHODS: Gross and microscopic features in 27 autopsies were analyzed with special attention to the heart and the vasculature of all organs. Findings were correlated with clinical features and ante-mortem investigations. Their contribution towards mortality was assessed. RESULTS: Valvar lesions were the commonest cardiac lesions noted with non-bacterial thrombotic endocarditis in nine (33.33%), valvar thickening in two (7.41%), Libman-Sacks endocarditis and infective endocarditis in one (3.70%) each. Myocarditis and myocardial scarring were seen in 10 (37.03%) and seven (25.92%) cases, respectively. Fibrinous pericarditis was noted in seven (25.92%). Thromboses/embolism, vasculitis and severe coronary atherosclerosis were seen in nine (33.33%), five (18.52%) and one (3.70%) subjects, respectively. Renal disease [13, 48.14%] and cardiovascular manifestations [8, 29.62%] were the leading causes of death in our patient population. CONCLUSION: CVD contributes significantly to the mortality in patients with SLE in India. It is second only to renal disease in this regard.

Thymoma: a pathological study of 50 cases.

BACKGROUND: A combination of epithelial cells and lymphocytes results in a varied histomorphology of thymomas and consequent varied classification systems. AIM: To correlate the Marino and Muller-Hermelink (MMH) classification with the invasive behaviour of thymomas. SETTING AND DESIGN: Retrospective analysis. MATERIALS AND METHOD: Thymomas encountered in the past 21 years were re-classified with the MMH classification and correlated with Masaoka's staging and clinical presentation. RESULTS: The thymomas formed 91% of the primary thymic epithelial tumours. Predominantly cortical thymomas (n=21) and cortical thymomas (n=22) were the common subtypes and 60% and 77% of these, respectively, were in stages II or III. Cystic change, necrosis or haemorrhage played no role in predicting invasive behaviour. Cortical epithelium correlated well with the presence of para-thymic syndromes, especially myasthenia gravis. CONCLUSION: MMH classification is easy to apply. Cortical thymomas in stage I should be followed up for possible recurrence.

Invasive pulmonary aspergillosis: A study of 39 cases at autopsy.

BACKGROUND: Aspergillus is a common cause of invasive mycosis, especially in immunocompromised or immunosuppressed individuals. AIMS: To study the incidence of invasive pulmonary aspergillosis and evaluate the predisposing factors and clinico-pathological manifestations. SETTINGS AND DESIGN: Retrospective analysis of autopsy material from a tertiary care hospital. MATERIAL AND METHODS: All autopsies performed over a 12-year period were reviewed and cases with invasive aspergillosis were analysed with respect to their clinical presentation, predisposing factors, gross and histological features, complications and causes of death. RESULTS: Among a total of 20475 autopsies performed in 12 years, 39 patients (0.19 %) had invasive pulmonary aspergillosis. There were 28 males and 11 females. Their ages ranged from five months to 67 years. Dyspnoea, fever, cough with mucopurulent expectoration, chest pain and haemoptysis were commonly encountered symptoms. Forty-one per cent of the patients had no respiratory symptoms. Fungal aetiology was not entertained clinically in any of the patients. The major underlying conditions were prolonged antibiotic therapy, steroid therapy, and renal transplantation, often associated with underlying lung diseases. Pneumonia, abscesses, vascular thrombosis and infarction were common findings at autopsy. Antecedent tuberculosis, mucormycosis, Pneumocystis carinii pneumonia and Cytomegalovirus infection were also present. In most cases, death was related to extensive pulmonary involvement or fungal dissemination. CONCLUSION: A diagnosis of invasive pulmonary aspergillosis should always be borne in mind whenever one is dealing with recalcitrant lung infections even with subtle immunosuppression. Radiological investigations and serologic markers can be utilised for confirmation and prompt therapy.

Angiosarcoma of the heart presenting as recurrent cardiac tamponade.

We present an interesting case of angiosarcoma of the heart presenting as recurrent percardial effusion and cardiac tamponade.

Blastopathies--symmetrical conjoined twins.

Conjoined twins are one of the rarest blastopathies, challenging the medical profession. The incidence in India is reported to be 1:60,000 pregnancies. This report records two cases of female conjoined twins. The sharing of various organs and complex cardiac anomalies encountered were very fascinating. Omphalopagus conjoined twins had only three lower extremeties, a common gastro-intestinal tract beyond the terminal ileum, crossed ectopia, hypoplastic kidneys and bicornuate uterus. The heart showed dextrocardia, A.S.D. and V.S.D. No attempt was made for surgical separation in this case. The second case was a thoracopagus, with the heart showing V.S.D., cor triatrium dexter with a posterior venous chamber. Surgical separation was attempted, but was unsuccessful. Both cases showed a single fused liver. The need for antenatal diagnosis and successful separation with good pre- and post-operative management is stressed.

Blalock Taussig type shunt palliation for cyanotic infants and children.

In a period of ten years from January 1, 1979 to December 31, 1988, 54 cyanotic patients weighing less than 10 kg underwent shunt operations of Blalock Taussig type. The indications were hypercyanotic spells, failure to thrive and pulmonary arteries being too small for safe total collection. The commonest diagnosis was tetralogy of Fallot (63%). Thirty-three (64%) patients were older than 1 year but still weighed less than ten kg. Mortality was 16.67% (70% C.L. 8.94-26.60). During follow-up, there were 4(7%) late deaths. During the same period, 134 patients less than 5 years age came to autopsy without having any cardiological or surgical intervention. Ninety-seven (72.4%) of these deaths were due to cardiac causes. In order to save their lives, early identification is necessary which highlights the importance of parent and primary physician education.

Pleomorphic intimal sarcoma of pulmonary artery. A case report.

A rare case of pleomorphic intimal sarcoma of pulmonary trunk is reported. The patient presented with symptoms of right ventricular out flow tract (RVOT) obstruction. Metastatic deposits were seen in lungs, diaphragm and thyroid. Bronchial mucosal involvement was also seen. The tumour showed multicentric origin and on electron microscopic examination in a particular cell line was seen.

Familial hypercholesterolaemia--report of two cases.

Two young female patients aged 12 and 20 years with homozygous familial hypercholesterolaemia are presented with autopsy reports. Both had extensive coronary artery disease with myocardial infarction and tendon xanthomas. The first case had additional aortic stenosis.

Collision tumour of stomach.

Collision tumour of the stomach is exceedingly rare, with only four previous reported instances in which adenocarcinoma of the stomach was found in association with a single carcinoid tumour. Our case with adenocarcinoma had multiple carcinoid tumours in the stomach.