Systemic vasculitis during the course of systemic sclerosis: report of 12 cases and review of the literature.

Publisher: Lippincott Williams & Wilkins Country of Publication: United States NLM ID: 2985248R Publication Model: Print Cited Medium: Internet ISSN: 1536-5964 (Electronic) Linking ISSN: 00257974 NLM ISO Abbreviation: Medicine (Baltimore) Subsets: MEDLINE

Original Publication: Hagerstown, Md : Lippincott Williams & Wilkins

Scleroderma, Systemic/*complications ; Vasculitis/*etiology; Adrenal Cortex Hormones/therapeutic use ; Adult ; Antibodies, Antineutrophil Cytoplasmic/immunology ; Antirheumatic Agents/therapeutic use ; Biomarkers/analysis ; Biopsy ; Cryoglobulinemia/drug therapy ; Cryoglobulinemia/immunology ; Cyclophosphamide/therapeutic use ; Female ; France ; Humans ; Middle Aged ; Penicillamine/therapeutic use ; Scleroderma, Systemic/drug therapy ; Scleroderma, Systemic/immunology ; Vasculitis/drug therapy ; Vasculitis/immunology

Although the presence of antineutrophil cytoplasmic antibodies (ANCA) has been reported in patients with systemic sclerosis (SSc), the association of SSc and systemic vasculitis has rarely been described. We obtained information on cases of systemic vasculitis associated with SSc in France from the French Vasculitis Study Group and all members of the French Research Group on Systemic Sclerosis. We identified 12 patients with systemic vasculitis associated with SSc: 9 with ANCA-associated systemic vasculitis (AASV) and 3 with mixed cryoglobulinemia vasculitis (MCV). In all AASV patients, SSc was of the limited type. The main complication of SSc was pulmonary fibrosis. Only 2 patients underwent a D-penicillamine regimen before the occurrence of AASV. The characteristics of AASV were microscopic polyangiitis (n = 7) and renal limited vasculitis (n = 2). Anti-myeloperoxidase antibodies were found in 8 of the 9 patients. The Five Factor Score was above 1 in 3 of the 9 patients. Of the 3 patients with MCV, Sjögren syndrome was confirmed in 2. We compared our findings with the results of a literature review (42 previously reported cases of AASV with SSc). Although rare, vasculitis is a complication of SSc. AASV is the most frequent type, and its diagnosis can be challenging when the kidney is injured. Better awareness of this rare association could facilitate earlier diagnosis and appropriate management to reduce damage.

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0 (Adrenal Cortex Hormones)
0 (Antibodies, Antineutrophil Cytoplasmic)
0 (Antirheumatic Agents)
0 (Biomarkers)
8N3DW7272P (Cyclophosphamide)
GNN1DV99GX (Penicillamine)

Date Created: 20121225 Date Completed: 20130312 Latest Revision: 20211021

20240829

PMC5370746

10.1097/MD.0b013e31827781fd

23263715