An autopsy case of progressive systemic sclerosis with proliferation of Niemann-Pick cell-like cells leading to liver fibrosis

• Published in: Kanzo Vol. 36; no. 2; pp. 107 - 110
• Main Authors: KAYO, Ryotaro, FUKUNISHI, Keiichi, SHIBAYAMA, Yuro, MATSUMIYA, Teisuke, ARAKI, Masato, URANO, Toru, ASAKA, Shosaku, ODA, Kosaku
• Format: Journal Article
• Language: Japanese
• Published: The Japan Society of Hepatology 1995
• ISSN: 0451-4203; 1881-3593
• DOI: 10.2957/kanzo.36.107

We report an autopsy case, a 50-year-old man, of progressive systemic sclerosis associated with proliferation of Niemann-Pick cell-like cells and liver fibrosis. In the present case, marked proliferation of foamy macrophages was seen in the spleen, liver, bone marrow etc. These cells had cytoplasmic vacuoles stained with oil red O and Nile blue. Ultrastructurally, the vacuoles contained concentric myelin-like materials, which are thought to be derived from phospholipids. Such cells resemble Niemann-Pick cells in Niemann-Pick disease. It has been recently reported that foamy macrophages having characteristics of Niemann-Pick cells, Niemann-Pick cell-like cells, are found in some autoimmune disorders. Accordingly, the appearance of Niemann-Pick cell-like cells in the present case may be related to progressive systemic sclerosis. In the liver, these Niemann-Pick cell-like cells were recognized as proliferation of swollen Kupffer cells. The swollen Kupffer cells narrowed the sinusoids mainly in the peripheral zone and led to atrophy and disappearance of hepatocytes and fibrosis without inflammatory cell reaction. Portal hypertension might be resulted from sinusoidal circulatory disturbance due to swollen Kupffer cells, periportal fibrosis and an increase in splenic blood flow related to splenomegaly.