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 glo-merulosclerosis). 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 drug-induced 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.