Nonspecific interstitial pneumonia with abundant IgG4-positive cells infiltration, which was thought as pulmonary involvement of IgG4-related autoimmune disease.
Recently, great attention has been drawn to IgG4-related diseases such as autoimmune pancreatitis (AIP) sclerosing sialadenitis, retroperitoneum fibrosis, sclerosing cholangitis. IgG4-related diseases are characterized by high serum IgG4 concentrations, sclerosing inflammation with numerous IgG4-positive plasma cells, and steroid sensitivity irrespective of their organs of origin. In this report, we describe a case of nonspecific interstitial pneumonia, in which possible involvement of IgG4 was suggested. The patient was 59-year-old man, who was found to have bilateral interstitial pneumonia. Laboratory tests revealed that he had antinuclear antibody and a high serum IgG4 concentration. Pathological examination of the video-assisted thoracic surgery biopsy taken from the right lower lobe showed interstitial thickening associated with lymphoplasmacytic infiltration containing many IgG4-positive plasma cells. He was effectively treated by corticosteroid. The present case had many clinical and clinicopathologic similarities to systemic IgG4-related autoimmune disease. There have been no descriptions on isolated interstitial pneumonia with IgG4-positive plasma cell infiltration. This case suggested that IgG4-related disorders could also occur in the lung, and interstitial pneumonia may be a pulmonary manifestation of systemic IgG4-related autoimmune disease.
Takato
Hazuki
H
Department of Respiratory Medicine, Kanazawa University Graduate School of Medical Science, Kanazawa, Japan.
Yasui
Masahide
M
Ichikawa
Yukari
Y
Fujimura
Masaki
M
Nakao
Shinji
S
Zen
Yoh
Y
Minato
Hiroshi
H
D001327:Autoimmune Diseases / Q000276:immunology / Q000473:pathology; D003937:Diagnosis, Differential; D006801:Humans; D007074:Immunoglobulin G / Q000096:biosynthesis*; D017563:Lung Diseases, Interstitial / Q000276:immunology* / Q000473:pathology*; D008297:Male; D008875:Middle Aged
D007074:Immunoglobulin G