A case of Sjögren syndrome complicated with lymphoproliferative disorder and idiopathic CD4 positive lymphocytopenia
Yoshiaki Kinoshitaa Atsuhiko Sakamotoa Yasushi Ikutab Kazuki Tamurab Kouko Hidakaa
aDepartment of Respiratory Medicine, National Hospital Organization Kokura Medical Center
bDepartment of Thoracic Surgery, National Hospital Organization Kokura Medical Center
A 55-year-old man was admitted to our hospital complaining of dry cough and fever. Computed tomography showed small nodules in the bilateral upper lobe and mediastinal lymphadenopathies. Thoracoscopic lung biopsy specimens showed findings consistent with lymphoproliferative disorder. He met the criteria of Sjögren's syndrome and idiopathic CD4 positive lymphocytopenia, and then suffered from recurring pneumothorax and respiratory failure. Systemic steroid therapy was started; however; he died of disseminated cryptococcosis. Lymphoproliferative disorder and idiopathic CD4 positive lymphocytopenia are uncommon disorders that are complicated with autoimmune disease. Moreover, the association in the present case between Cryptococcus infection and idiopathic CD4 positive lymphocytopenia was indicated.
Sjögren's syndrome Lymphoproliferative disorder Follicular bronchiolitis Idiopathic CD4 positive lymphocytopenia Disseminated cryptococcosis
Received 1 Jun 2012 / Accepted 21 Sep 2012
AJRS, 2(3): 228-232, 2013