common variable immunodeficiency;
corticosteroid;
CTLA-4;
deficiency;
granulomatous-lymphocytic interstitial lung disease;
video-assisted thoracoscopic surgery;
COMMON VARIABLE IMMUNODEFICIENCY;
DIAGNOSIS;
D O I:
10.2169/internalmedicine.0076-22
中图分类号:
R5 [内科学];
学科分类号:
1002 ;
100201 ;
摘要:
Common variable immunodeficiency (CVID) causes granulomatous-lymphocytic interstitial lung disease (GLILD) and has a poor prognosis. We herein report a case of GLILD in a 49-year-old woman with CTLA-4 deficiency-associated CVID. The patient presented with dyspnea that had worsened over the past two years. A laboratory examination revealed hypoglobulinemia and pancytopenia. Chest computed tomography showed diffuse infiltrative and granular shadows in the bilateral interstitium. A flow cytometric analysis of blood cells and genetic testing confirmed CTLA-4 deficiency. We performed video-assisted thoracoscopic surgery for the pathological diagnosis of GLILD and to exclude infection and malignancy. Corticosteroid treatment successfully improved the condition of the patient.
机构:
Med Coll Wisconsin, Med Cardiol, Milwaukee, WI 53226 USAMed Coll Wisconsin, Med Cardiol, Milwaukee, WI 53226 USA
Hoffmann, Brian R.
Vallejos, Andrew
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机构:
Med Coll Wisconsin, Physiol, Milwaukee, WI 53226 USAMed Coll Wisconsin, Med Cardiol, Milwaukee, WI 53226 USA
Vallejos, Andrew
Greene, Andrew S.
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机构:
Med Coll Wisconsin, Physiol, Milwaukee, WI 53226 USAMed Coll Wisconsin, Med Cardiol, Milwaukee, WI 53226 USA
Greene, Andrew S.
Routes, John M.
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h-index: 0
机构:
Med Coll Wisconsin, Pediat, Milwaukee, WI 53226 USA
Childrens Hosp Wisconsin, Milwaukee, WI 53201 USAMed Coll Wisconsin, Med Cardiol, Milwaukee, WI 53226 USA