A Patient with Diffuse Cutaneous Systemic Sclerosis Complicated by Antineutrophil-cytoplasmic Antibody-associated Vasculitis Exhibiting Honeycomb Lung without Volume Loss

Yamashita, Hiroyuki; Takahashi, Yuko; Kaneko, Hiroshi; Kano, Toshikazu; Matsubara, Akio

doi:10.2169/internalmedicine.53.1599

Cited by 6 publications

(3 citation statements)

References 13 publications

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“…The clinical significance of ANCA in SSc patients who do not manifest AAV is controversial. An association between ANCA in SSc and ILD has been suggested [12, 20–22]. However, this has not been consistently reported in all case series [15].…”

Section: Introductionmentioning

confidence: 99%

Significance of anti-neutrophil cytoplasmic antibodies in systemic sclerosis

et al. 2019

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Background Up to 12% of patients with systemic sclerosis (SSc) have anti-neutrophil cytoplasmic antibodies (ANCA). However, the majority of these patients do not manifest ANCA-associated vasculitis (AAV) and the significance of ANCA in these patients is unclear. The aim of this study is to determine the prevalence of ANCA in a well-characterised SSc cohort and to examine the association between ANCA and SSc clinical characteristics, other autoantibodies, treatments and mortality. Methods Clinical data were obtained from 5 centres in the Australian Scleroderma Cohort Study (ASCS). ANCA positive was defined as the presence of any one or combination of cytoplasmic ANCA (c-ANCA), perinuclear ANCA (p-ANCA), atypical ANCA, anti-myeloperoxidase (anti-MPO) or anti-proteinase-3 (anti-PR3). Associations of demographic and clinical features with ANCA were investigated by logistic or linear regression. Survival analysis was performed using Kaplan-Meyer curves and Cox regression models. Results Of 1303 patients, 116 (8.9%) were ANCA positive. Anti-PR3 was more common than anti-MPO (13.8% and 11.2% of ANCA-positive patients, respectively). Only 3 ANCA-positive patients had AAV. Anti-Scl-70 was more common in ANCA positive vs ANCA negative (25% vs 12.8%, p < 0.001), anti-MPO positive vs anti-MPO negative (38.5% vs 13.6%, p = 0.006) and anti-PR3 positive vs anti-PR3 negative patients (44.4% vs 13.4%, p < 0.001). A higher prevalence of interstitial lung disease (ILD) was found in the ANCA positive (44.8% vs 21.8%, p < 0.001) and the anti-PR3 positive groups (50.0% vs 23.4%, p = 0.009). In multivariable analysis, ANCA-positive status remained associated with ILD after adjusting for anti-Scl-70 antibodies. Pulmonary embolism (PE) was more common in ANCA-positive patients (8.6% vs 3.0%, p = 0.002) and anti-PR3-positive patients (16.7% vs 3.3%, p = 0.022). ANCA-positive status remained associated with PE in a multivariable analysis adjusting for anti-phospholipid antibodies. Kaplan-Meier analysis revealed increased mortality in ANCA-positive patients ( p = 0.006). In Cox regression analysis, ANCA was associated with increased mortality, after adjusting for age and sex. Conclusions ANCA is associated with increased prevalence of ILD and PE in SSc. ANCA should be tested in SSc, as it identifies individuals with worse prognosis who require close monitoring for adverse outcomes.

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Section: Introductionmentioning

confidence: 99%

Significance of anti-neutrophil cytoplasmic antibodies in systemic sclerosis

et al. 2019

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“…We identified six papers describing the clinical features of 12 SSc patients with ANCA‐associated neuropathy (Table 1). 2,3,5–8 In all the reported cases, the SSc diagnosis preceded the ANCA‐associated neuropathy diagnosis. Positive results for anti‐Scl‐70 antibody were observed in five patients (5/12).…”

Section: Discussionmentioning

confidence: 98%

ANCA‐associated neuropathy in systemic sclerosis: A case report and review of literature

Takenaka

Takeichi

Nishi

et al. 2021

J Cutaneous Imm & Allergy

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Systemic sclerosis (SSc) is a multi‐system autoimmune disease. Anti‐neutrophil cytoplasmic antibodies (ANCA) are autoantibodies directed against enzymes found within primary granules of neutrophils and lysosomes in monocytes. Although up to 12% of SSc patients have ANCA, only a minority of these patients develop an overlap syndrome with ANCA‐associated vasculitis. We summarize previous reports on SSc patients with ANCA‐associated neuropathy. In all the reported cases, the SSc diagnosis preceded the ANCA‐associated neuropathy diagnosis. Seven of the eight patients with limited cutaneous SSc had interstitial lung disease (ILD). Thus, patients with ANCA‐associated neuropathy in lSSc may be prone to complication with ILD.

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“…reported a case of AAV in 72-year-old lady with diffuse cutaneous SSc with cellular crescent on renal biopsy and mononeuritis multiple. [ 2 ] In another study with 12 patients of systemic vasculitis and associated SSc, AAV was found in nine patients. Renal limited vasculitis was found in only two out of these nine patients.…”

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confidence: 99%