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A 49-year-old female was admitted to our hospital because of worsening of congestive heart failure on November 2000 in a state after insertion of permanent pacemaker for complete atrioventricular block in 1986, followed by a clinical history of chronic heart failure due to dilated cardiomyopathy. After admission, her general condition had been improved, but, she had massive hemoptysis suddenly and died on February 2001. At autopsy, noncaseating granulomas were observed scattering in lungs, liver and spleen, not associated with any infectious lesions, therefore indicating systemic sarcoidosis. In lungs, granulomatous arteritis in small- and medium-sized muscular arteries associated with disputation of the media and elastic laminae were observed, suggesting the direct cause of hemoptysis. This is the extremely rare case of pulmonary arteritis with systemic sarcoidosis resulting the death from massive hemoptysis.

We describe a case of a 61-year-old woman with amyopathic dermatomyositis (ADM), who developed rapidly progressive interstitial pneumonia and died of respiratory failure. An autopsy revealed interstitial pneumonia with diffuse alveolar damage, associated with infiltration of T cells, mostly positive for CD 8. The alveolar lining epithelial cells manifested the remarkable expression of immediate early/early antigen of human cytomegalovirus (HCMV). Moreover, the extract of the lung was transmittable of HCMV infection to cultured human embryo-fibroblasts in vitro. On the other hand, in the semi-quantitative analysis of HCMV genome, using laser-assisted microdissection, followed by PCR method, the genomic DNA in the alveolar lining epithelial cells was little detected in this case, although it was remarkable in the case of immunodeficiency with cytomegalovirus pneumonia. This case may be important to know the role of the immune response of host to HCMV infection on the development of rapidly progressive interstitial pneumonia.

A 43-year-old man was admitted to a hospital because of acute dyspnea and nocturnal orthopnea. Echocardiogram and chest CT showed the dilation of thoracic aorta from the root to ascending portion. On the third hospital day, he died suddenly. At autopsy, the cause of death was indicated to be a tear of an aortic valve due to a rupture of the aneurysm of Valsalva's sinus, followed by acute aortic regurgitation and acute cardiac insufficiency. Histopathological findings of thoracic aorta revealed mesoaortitis, characterized by patchy destruction of the media with a moth-eaten appearance of the medial elastic laminae and a microgranuloma formation, a perivascular mononuclear cell infiltration of the vasa vasorum, and a fibrous thickening of the intima and adventitia. However, there were no abnormalities in main branches of aorta and abdominal aorta, and no systemic vasculitis. This case is a rare one in the clinical course, and may be important to be differentiated from other cases with aortitis, especially Takayasu arteritis and syphilitic aortitis.

Segmental Mediolytic Arteritis (SMA) is a very rare arterial lesion which is limited in adults to the involvement of the intra-abdominal muscular arteries. The pathology is characterized by segmental disruption of the arterial media which leads segmental mediolysis, with subsequent dissecting aneurysm or rupture. A 73-year-old man was admitted to a hospital because of high fever, general fatigue and weight loss. These symptoms were resistant to antibiotic therapy, and soon after, renal insufficiency developed. Three months after the onset of symptoms, he died suddenly of hemorrhagic shock. Autopsy revealed rupture of the splenic artery and systemic necrotizing arteritis in the small-sized arteries of liver, pancreas and kidneys, as well as in the connective tissues around the adrenal glands. Histopathology of the splenic artery was consistent with SMA, and that of the systemic vascular lesions, with microscopic polyarteritis nodosa. Focal glomerular lesions characteristic of crescentic and/or granulomatous glomerulonephritis were present. A developmental mechanism for SMA is discussed with respect to this case together with a review of previous reports of this disease.

Almost all of intractable vasculitis have been thought to develop mainly in immunological mechanisms, based on the data from several animal models previously established. Serum sickness models, microbe-induced models and others have contributed to supporting this idea. These models have furthermore indicated that vasculitis could be generated by exogenous factors in normal individuals, and that vasculitis might be explained in monism. However, recent studies in murine lupus suggest that intractable vasculitis requires endogenous factors involving retroviruses and/or genetic background under the control of polygene system. In this review, although not comprehensive, significance of background genes in vasculitis will be focused.