---
title: "Systemic sclerosis complicated by MPO-positive vasculitis with multi-site hemorrhage"
id: "pubmed-42880933"
canonical_url: "https://medichelpline.com/clinical-feed/pubmed-42880933"
content_type: "clinical_feed_article"
specialty: "Critical Care"
source_name: "PubMed / NCBI"
source_url: "https://pubmed.ncbi.nlm.nih.gov/42880933/"
published_at: "2026-10-18T00:00:00.000Z"
evidence_level: "Journal Article"
license: "CC-BY-NC-4.0 / Informational Use"
---
# Systemic sclerosis complicated by MPO-positive vasculitis with multi-site hemorrhage
## Provenance & Clinical Metadata
- **Canonical URL:** https://medichelpline.com/clinical-feed/pubmed-42880933
- **Specialty:** [Critical Care](https://medichelpline.com/clinical-feed/critical-care.md)
- **Primary Source:** PubMed / NCBI
- **Source URL:** [Original Journal Publication](https://pubmed.ncbi.nlm.nih.gov/42880933/)
- **Published At:** 2026-10-18T00:00:00.000Z
- **Evidence Rating:** Journal Article
## Executive GIST (TL;DR)
- This is a single-patient case report of a 56-year-old woman with long-standing **systemic sclerosis** (20 years of Raynaud phenomenon, fingertip and facial skin sclerosis) who developed progressive respiratory and renal symptoms. - Initial workup documented dry cough, exertional dyspnea, moderate anemia, impaired renal function, proteinuria, hematuria and positive anti-topoisomerase I (anti-SCL-70). - Chest CT showed interstitial fibrosis in both lower lungs consistent with SSc-related lung disease. - The patient was initially managed with low-dose prednisone and nifedipine while chronic renal injury was attributed to SSc. - Rapid renal deterioration occurred during treatment; immunology testing became positive for anti-myeloperoxidase (**MPO**) antibody and renal biopsy demonstrated **crescentic glomerulonephritis**. - The diagnosis was revised to **systemic sclerosis** complicated by **anti-neutrophil cytoplasm antibody-associated vasculitis (AAV)** with crescentic nephritis and interstitial lung disease. - High-dose methylprednisolone (500 mg then escalated to 1 g for 3 days), plasma exchange and cyclophosphamide were used for presumed severe AAV; one day after initial pulse the patient developed hemoptysis consistent with **alveolar hemorrhage**. - During intensified therapy the patient had an episode of coffee-colored vomiting and melena (gastrointestinal hemorrhage); steroids were reduced and plasma exchange plus cyclophosphamide continued with acid suppression. - The acute condition initially improved and the patient was discharged, but she later developed pulmonary infection, recurrent gastrointestinal bleeding and cerebral hemorrhage during anti-infective treatment and ultimately died. - The report emphasizes that coexistence of **SSc** and **AAV** is rare, tends to involve the kidneys as crescentic glomerulonephritis, and that combined alveolar or cerebral hemorrhage carries high mortality; the source did not report detailed timelines beyond those summarized, nor full dosing schedules for all therapies beyond the methylprednisolone pulses noted.
## Clinical Analysis & Structured Key Points
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Affiliations Expand ### Affiliation * 1 Department of Immunology, Mianyang Central Hospital, Mianyang 621000, Sichuan, China. * PMID: **42880933** Free article Item in Clipboard Case Reports # [Systemic sclerosis with anti-human neutrophil cytoplasm antibody associated vasculitis: A case report] [Article in Chinese] Li Yang et al. Beijing Da Xue Xue Bao Yi Xue Ban. 2026. Free article Show details Display options Display options Format Abstract PubMed PMID Beijing Da Xue Xue Bao Yi Xue Ban Actions * [ Search in PubMed ](https://pubmed.ncbi.nlm.nih.gov/?term=%22Beijing+Da+Xue+Xue+Bao+Yi+Xue+Ban%22%5Bjour%5D&sort=date&sort_order=desc) * [ Search in NLM Catalog ](https://www.ncbi.nlm.nih.gov/nlmcatalog?term=%22Beijing+Da+Xue+Xue+Bao+Yi+Xue+Ban%22%5BTitle+Abbreviation%5D) * [ Add to Search ](https://pubmed.ncbi.nlm.nih.gov/42880933/) . 2026 Oct 18;58(5):1084-1089. ### Authors [Li Yang](https://pubmed.ncbi.nlm.nih.gov/?term=Yang+L&cauthor_id=42880933)[ 1 ](https://pubmed.ncbi.nlm.nih.gov/42880933/#short-view-affiliation-1 "Department of Immunology, Mianyang Central Hospital, Mianyang 621000, Sichuan, China."), [Jinmei Zou](https://pubmed.ncbi.nlm.nih.gov/?term=Zou+J&cauthor_id=42880933)[ 1 ](https://pubmed.ncbi.nlm.nih.gov/42880933/#short-view-affiliation-1 "Department of Immunology, Mianyang Central Hospital, Mianyang 621000, Sichuan, China.") ### Affiliation * 1 Department of Immunology, Mianyang Central Hospital, Mianyang 621000, Sichuan, China. * PMID: **42880933** Item in Clipboard Full text links Cite Display options Display options Format Abstract PubMed PMID Linked Discoveries ## 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. **Keywords:** Alveolar hemorrhage; Anti-human neutrophil cytoplasm antibody-associated vasculitis (AAV); Cerebral hemorrhage; Cresentic glomerulonephritis; Systemic sclerosis. 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