Can antineutrophil cytoplasmic antibody positivity at diagnosis predict the poor outcomes of Sjögren’s syndrome?

Lee, Soo Bin; Choi, Hyang Suk; Kim, Minyoung Kevin; Jung, Seung Min; Song, Jason Jungsik; Park, Yong Bum; Lee, Sang Won

doi:10.1007/s00296-019-04476-5

Cited by 4 publications

(2 citation statements)

References 22 publications

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“…Conversely, in pSjS there are cases in which ANCA is already elevated at the time of onset. However, it is uncommon to develop slowly progressing AAV afterwards [12]. As shown here, ANCA can become elevated during the disease course, and there are cases of smoldering or slowly progressing AAV in patients with pSjS.…”

Section: Discussionmentioning

confidence: 61%

A case of smoldering antineutrophil cytoplasmic antibody-associated vasculitis development during the course of primary Sjögren’s syndrome

et al. 2021

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Various forms of glomerular lesions have been described in primary Sjögren's syndrome (pSjS); however, myeloperoxidase (MPO)-antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is rarely reported, and the disease onset and clinical course of ANCA-associated vasculitis (AAV) complicated by pSjS are not well understood. A 51-year-old woman was referred to our hospital because of mild proteinuria and microscopic hematuria. She fulfilled the classification criteria for pSjS. We performed a kidney biopsy; however, it revealed no characteristic findings for pSjS, vasculitis, or other autoimmune diseases, including systemic lupus erythematosus. After 9 months, urinalysis abnormalities worsened and renal function was slowly declining, and ANCA was found to be positive. A second kidney biopsy was performed, revealing MPO-ANCA-associated pauci-immune segmental necrotizing glomerulonephritis with crescent formation. Even though immunofluorescence microscopy did not reveal any positive findings, additional electron microscopy demonstrated the presence of mesangial electron-dense deposits in both kidney biopsies. Based on kidney biopsy results and sequential serum ANCA measurements, we considered that smoldering ANCA-associated vasculitis had developed in this patient as this can develop during the clinical course of pSjS. She responded well to steroid therapy. Serum measurement, especially perinuclear, ANCA levels can be useful in patients with pSjS to detect the onset of ANCA-associated vasculitis, even in the absence of acute renal deterioration or severe urinary abnormalities.

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Section: Discussionmentioning

confidence: 61%

A case of smoldering antineutrophil cytoplasmic antibody-associated vasculitis development during the course of primary Sjögren’s syndrome

et al. 2021

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“…Markers of higher disease activity, such as an elevated erythrocyte sedimentation rate, C-reactive protein, white blood cell count and lactate dehydrogenase, and low complement levels have also all been associated with the development of ILD in SjS patients [5 ▪ ]. Concomitant presence of antineutrophil cytoplasmic antibodies (ANCA) are related to ILD incidence, with a positive p-ANCA and MPO-ANCA additionally associated with lower ILD-free survival in SjS [8]. Autoantibodies against Ro/SSA antigens are a common serologic feature of SjS, and the two major antigens have weights of 52 kD and 60 kD.…”

Section: Sjogren's Syndromementioning

confidence: 99%

The other connective tissue disease-associated interstitial lung diseases: Sjogren's syndrome, mixed connective tissue disease, and systemic lupus erythematosus

Lee

Strek²

2021

Current Opinion in Pulmonary Medicine

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Purpose of reviewWe review the clinical manifestations of three less common connective tissue disease (CTD)-associated interstitial lung diseases (ILDs): Sjogren's syndrome (SjS), mixed CTD (MCTD), and systemic lupus erythematosus (SLE). Recent findingsSjS is classically associated with lymphocytic interstitial pneumonia and cystic lung disease, but the most common type of ILD in Sjogren's patients is nonspecific interstitial pneumonia. ILD is prevalent in MCTD and associated with worse survival. SLE-associated ILD, while rare, is more common in those with CTD overlap syndromes. Regardless of underlying cause, a subset of patients with fibrotic CTD-associated ILD develop a progressive course for which antifibrotic agents and lung transplantation should be considered. SummaryAn understanding of the characteristics of ILD in SjS, MCTD, and SLE is important for the pulmonary specialist. Future research should identify risk factors for progression and develop additional treatment modalities for both CTD-related autoimmune features and progressive ILD.

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Serum ANCA as Disease Biomarkers: Clinical Implications Beyond Vasculitis

Folci

Ramponi

Solitano

et al. 2021

Clinic Rev Allerg Immunol

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Can antineutrophil cytoplasmic antibody positivity at diagnosis predict the poor outcomes of Sjögren’s syndrome?

Cited by 4 publications

References 22 publications

A case of smoldering antineutrophil cytoplasmic antibody-associated vasculitis development during the course of primary Sjögren’s syndrome

A case of smoldering antineutrophil cytoplasmic antibody-associated vasculitis development during the course of primary Sjögren’s syndrome

The other connective tissue disease-associated interstitial lung diseases: Sjogren's syndrome, mixed connective tissue disease, and systemic lupus erythematosus

Serum ANCA as Disease Biomarkers: Clinical Implications Beyond Vasculitis

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