A case of systemic sclerosis complicated by idiopathic portal hypertension: case report and literature review

Kenji TakagiDivision of Rheumatology, Department of Internal Medicine (Omori), Toho University School of Medicine, 6-11-1 Omori-Nishi, Ota-ku, Tokyo 143-8541, JapanCorrespondence[email protected]

Shinichiro NishioDivision of Rheumatology, Department of Internal Medicine (Omori), Toho University School of Medicine, 6-11-1 Omori-Nishi, Ota-ku, Tokyo 143-8541, Japan

Kimiko AkimotoDivision of Rheumatology, Department of Internal Medicine (Omori), Toho University School of Medicine, 6-11-1 Omori-Nishi, Ota-ku, Tokyo 143-8541, Japan

Takumi YoshinoDivision of Rheumatology, Department of Internal Medicine (Omori), Toho University School of Medicine, 6-11-1 Omori-Nishi, Ota-ku, Tokyo 143-8541, Japan

Shinichi KawaiDivision of Rheumatology, Department of Internal Medicine (Omori), Toho University School of Medicine, 6-11-1 Omori-Nishi, Ota-ku, Tokyo 143-8541, Japan

Abstract

We encountered a 62-year-old woman who had systemic sclerosis (SSc) complicated by idiopathic portal hypertension (IPH). She had a 10-year history of scleroderma and Raynaud’s phenomenon. She also had pancytopenia, splenomegaly, and esophageal varices. Treatment with prednisolone and endoscopic variceal ligation resulted in improvement of her symptoms. According to our literature review, the prognosis of patients with SSc complicated by IPH is relatively poor. However, the factors that predict outcome of these patients have not been elucidated.

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A case of systemic sclerosis complicated by idiopathic portal hypertension: case report and literature review

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