An autopsy case of myocardial sarcoidosis.
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We present a case of predominantly myocardial sarcoidosis in a 38 year-old man. A second degree atrioventricular block was the clinical presentation, followed 2 years later by sudden cardiac arrest. Autopsy revealed large areas of myocardial scarring in the left ventricular wall unassociated with atherosclerotic coronary lesions; microscopy of these areas disclosed epithelioid granuloma with giant cells embedded in a fibrohyaline tissue. The insidious cardiac involvement in sarcoidosis often hinders an exact diagnosis in life, and prognosis without therapy is severe. Histopathologic diagnosis is made only by exclusion of other systemic granulomatous diseases.
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A patient was presented with severe congestive heart failure due to myocardial sarcoidosis diagnosed at autopsy. We found that the diagnosis of myocardial sarcoidosis is difficult when there is no other organ involvement.
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An autopsy case of sarcoidosis followed up for 27 years in a 48‐year‐old woman with pulmonary fibrosis is presented. Chest roentgenography demonstrated an interstitial pneumonia‐like pattern with gradual contraction of the upper lobes. Focal and extending fibrosis and hyalinizatlon were observed in various organs including the lung, lymph nodes, heart and liver. Most of the fibrosis was thought to be derived from solitary or confluent granuloma, showing hyalinized nodular, stellate or band‐like fibrosis. There was also another type of fibrosis, not derived from granuloma, manifested as fibrosing alveolitis in the lung, and diffuse fibrosis extending throughout the other organs. In the lymph nodes, chromogenic bodies (Hamazaki‐Wesenberg bodies) were seen. The process of fibrosis and the significance of chromogenic bodies in cases of chronic sarcoidosis are discussed. ACTA PATHOL JPN 38: 909 ∼ 920, 1988.
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Prognosis of 435 sarcoidosis patients and epidemiologic features were studied in 320 autopsy cases. About 80-90% of Stage I or II patients showed spontaneous remission. The prognosis of Stage III patient was poor and corticosteroid therapy seemed to be rather ineffective. The proportion of sarcoidosis autopsy, relative to total autopsy cases, appears to be increasing. The number of female cases was about 2 times larger than that of males. Only 43.4% of the total autopsy cases had been diagnosed clinical sarcoidosis. Over 5 times more sarcoidosis patients than clinically diagnosed were estimated to exist. Approximately one half of the sarcoidosis autopsy cases were related to cardiac sarcoidosis.
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Materials on heart sarcoidosis are presented on the basis of the literature analysis, morphological and electron microscopical examination of an autopsy observation. Heart sarcoidosis is characterized morphologically by granulomatous myocarditis with diffuse infiltration with lymphoid and macrophage cells, and marked destructive changes in muscle fibers. A damaging effect of the infiltrate cells on cardiomyocytes is shown.
Infiltration (HVAC)
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Pathological studies on sarcoidosis autopsy. II. Early change, mode of progression and death pattern
Three hundred and twenty autopsy cases of sarcoidosis in Japan were analyzed to determine the pathological changes in the early stage, the mode of progression in each organ and the changes in the final stage of the disease. The lung and the mediastinal lymph nodes were affected in most of the cases, while the lesions were limited to the lung and intrathoracic nodes in some of the cases. It was suspected that early changes developed in the lung and in the hilar, and then in the mediastinal lymph nodes. The progression of sarcoid granulomas in the lung was classified into three patterns: (i) probably of a disseminated hematogenous nature; (ii) of an interstitial lymphogenous nature; and (iii) of a local expansive nature. These three patterns were observed also in the heart. In the brain, perivascular granuloma formation was a prominent feature. In the other organs in which sarcoid lesions were not malignant nor disseminated and conglomerated, no interstitial patterns were observed. In chronic cases, repeated dissemination and particularly the interstitial spread of granulomatous changes led to a prominent interstitial fibrosis and dysfunction of the organs, finally resulting in death of the individual. In such longstanding cases, the mediastinal nodes deteriorated by hyalinous degeneration of the granulomas, and many active granulomas were formed in the intra‐abdominal or body surface lymph nodes. These lymph nodes were likely to continue supplying sensitized lymphocytes to the whole body. A persistence of active change in the lymph nodes and the lymphogenous spread of granulomas in organs would appear to be key factors in the prognosis of sarcoidosis.
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