Severe pulmonary hypertension associated with primary Sjögren's syndrome.
Severe pulmonary hypertension is one of the fetal complications in various connective tissue diseases. We report a case of severe pulmonary hypertension associated with primary Sjögren's syndrome. In a lung biopsy specimen, there were findings of intimal and medial hypertrophy with narrowing vessel lumina and plexiform lesions. Moreover, deposits of immunoglobulin M, immunoglobulin A and complement protein C1q were found in the pulmonary arterial walls. Although pulmonary hypertension was refractory to oral prostacyclin, steroid therapy improved the clinical and hemodynamic conditions. In the present case, the immunological etiology may be related to the mechanisms of pulmonary hypertension associated with Sjögren's syndrome.
Nakagawa
Naoki
N
First Department of Medicine, Asahikawa Medical College, Asahikawa.
Osanai
Shinobu
S
Ide
Hiroshi
H
Nishigaki
Yutaka
Y
Takahashi
Saori
S
Nakano
Hitoshi
H
Ohsaki
Yoshinobu
Y
Kikuchi
Kenjiro
K
Tokusashi
Yoshihiko
Y
Obata
Hiromi
H
D000328:Adult; D000936:Antigen-Antibody Complex / Q000378:metabolism; D015922:Complement C1q / Q000378:metabolism; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000276:immunology* / Q000378:metabolism / Q000473:pathology; D007070:Immunoglobulin A / Q000378:metabolism; D007075:Immunoglobulin M / Q000378:metabolism; D007150:Immunohistochemistry; D008168:Lung / Q000473:pathology; D011239:Prednisolone / Q000627:therapeutic use; D011651:Pulmonary Artery / Q000378:metabolism / Q000473:pathology; D012859:Sjogren's Syndrome / Q000150:complications* / Q000378:metabolism
D000936:Antigen-Antibody Complex; D005938:Glucocorticoids; D007070:Immunoglobulin A; D007075:Immunoglobulin M; D015922:Complement C1q; D011239:Prednisolone