Beijing Da Xue Xue Bao Yi Xue Ban. 2026 Oct 18;58(5):1084-1089.
ABSTRACT
Systemic sclerosis (SSc) is an autoimmune disease involving microvessels and a wide range of connective tissues. Anti-human neutrophil cytoplasm antibody-associated vasculitis (AAV) is a primary, systemic necrotizing small vasculitis. The coexistence of AAV and SSc is rare. We report a case of SSc combined with AAV complicated with alveolar hemorrhage, gastrointestinal hemorrhage, and cerebral hemorrhage. The patient was a 56-year-old woman with a 20-year history of Raynaud phenomenon, skin sclerosis on the fingertip and face. She developed dry cough for 1 year and shortness of breath after activity for 1month. Blood routine test showed moderate anemia, blood biochemistry showed renal function damage, and urine routine test showed positive urine protein and occult blood. Immunological examination showed that anti-topoisomerase Ⅰ (anti-SCL-70) antibody was positive. Lung CT showed interstitial fibrosis in both lower lungs. Systemic sclerosis and chronic renal injury were diagnosed and she was treated with low-dose prednisone and nifedipine. During the treatment, the patient developed rapidly progressive renal insufficiency, immunological indicator showed positive anti-myeloperoxidase antibody (MPO), and renal pathology suggested crescentic glomerulonephritis. The renal damage was considered to be caused by vasculitis, and the diagnosis was modified as systemic sclerosis complicated with AAV, with crescentic nephritis and interstitial lung disease. She was treated with methylprednisolone 500 mg. One day later, the patient developed hemoptysis, alveolar hemorrhage was considered, and treatment was adjusted to methylprednisolone 1 g for 3 days, plasma exchange and cyclophosphamide. Three days later, she had vomiting coffee-colored gastric contents and melena. Gastrointestinal hemorrhage was considered, and methylprednisolone was reduced to 80 mg. Plasma exchange and cyclophosphamide treatment were continued, supplemented by acid suppression. The patient's condition was finally controlled, improved and discharged. One month later, the patient developed cough and sputum, and lung CT showed pulmonary infection. During anti-infection treatment, the patient developed gastrointestinal hemorrhage again, accompanied by cerebral hemorrhage, and finally died. This case shows that the incidence of SSc combined with AAV is dangerous, mainly with kidney involvement, manifested as crescents glomerulonephritis. When combined with alveolar hemorrhage, cerebral hemorrhage, the mortality rate is high. Clinicians should pay attention to it, early diagnosis and active treatment should be taken seriously.
PMID:42880933