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Abstract

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Article in Japanese

Case Report

A sibling case of familial interstitial pneumonia, including ANCA-associated vasculitis

Taro Okabea  Hidefumi Koha  Nobufumi Kamiishia  Akihiko Sudoa  Hidenori Takahashia  Atsushi Chiyotania  Yoshishige Kimurab  Atsushi Tajimab  Yasuhiro Ohkuboc  Akira Umedad  Makio Mukaie 

aDivision of Pulmonary Medicine, Department of Internal Medicine, Saiseikai Utsunomiya Hospital
bDepartment of General Thoracic Surgery, Saiseikai Utsunomiya Hospital
cDivision of Nephrology, Department of Internal Medicine, Saiseikai Utsunomiya Hospital
dDepartment of Pulmonary Medicine, International University of Health and Welfare, Shioya Hospital
eDepartment of Pathology, Keio University School of Medicine

ABSTRACT

Case 1: A 78-year-old woman was diagnosed as idiopathic pulmonary fibrosis (IPF) at 62 years old and received medical follow-up. She was admitted to the hospital because of acute exacerbation of IPF as a result of viral infection. She passed away before need of intensive care. Histopathological findings of the specimen obtained by lung autopsy showed advanced usual interstitial pneumonia (UIP). Case 2: Bronchoscopy was performed on a 68-year-old woman for patchy ground-glass opacities. A lung biopsy specimen shows lymphocytic inflammation within the interstitium alveolar wall thickening. She was detected having renal insufficiency with high P-ANCA three years after the bronchoscopy. Renal biopsy revealed necrotizing crescentic glomerulonephritis. Finally, she was diagnosed as ANCA-associated vasculitis. Case 3. A 63-year-old man visited the hospital because of persistent cough and dyspnea. He underwent performance of video-assisted thoracic surgery and was diagnosed as UIP. Familial interstitial pneumonia (FIP) is estimated to be 0.5-2.0% of idiopathic interstitial pneumonia. To the best of our knowledge, this is the first report of FIP including ANCA-associated vasculitis and IPF.

KEYWORDS

Familial interstitial pneumonia  ANCA-associated vasculitis  Idiopathic pulmonary fibrosis 

Received 29 Aug 2012 / Accepted 31 Jan 2013

AJRS, 2(4): 365-369, 2013

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