Open AccessTo the issue of differential diagnosis of ANCA-positive generalized sarcoidosis and ANCA-associated polyangiitis
Author(s) -
Г. Б. Ковальский,
Б. М. Ариэль,
Vladimir Volchkov,
И. А. Данилова,
А. Г. Полиевец
Publication year - 2018
Publication title -
pulʹmonologiâ
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.126
H-Index - 6
eISSN - 2541-9617
pISSN - 0869-0189
DOI - 10.18093/0869-0189-2017-28-3-496-502
Subject(s) - medicine , granulomatosis with polyangiitis , pathology , sarcoidosis , vasculitis , microscopic polyangiitis , differential diagnosis , respiratory distress , autopsy , nephritis , disease , surgery
An autopsy case of a female patient of 65 years old was reported in the article. This case demonstrated clinical and anatomical features of chronic generalized sarcoidosis with predominant lesion of the lungs (extended bilateral fibrosis) and the kidneys (interstitial nephritis with segmental glomerulosclerosis). Long-term steroid therapy resulted in a significant slowing progression of the disease. This case was characterized by a prominent vasculitis which caused the misdiagnosis of ANCA- associated polyangiitis. Severe anemia and secondary immunodeficiency that were partially druginduced due to the long-term steroid therapy were other manifestations of this disease. The advanced disease was complicated by lower respiratory tract infection, acute respiratory distress-syndrome, and multiple organ dysfunction that caused the patient’s death. A comprehensive pathological investigation including immunohistochemistry had the crucial role for the final diagnosis.