ANCA in systemic sclerosis, when vasculitis overlaps with vasculopathy: a devastating combination of pathologies

Hughes, Michael; Kahaleh, Bashar; Denton, Christopher P.; Mason, Justin C.; Matucci‐Cerinic, Marco

doi:10.1093/rheumatology/keab278

Cited by 11 publications

(12 citation statements)

References 47 publications

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“…There may be also positivity for anti-dsDNA antibodies or antineutrophil cytoplasmic antibodies (ANCA), which may indicate a possible evolution to another connective disease even in the absence of clinical changes [ 43 , 44 , 45 ]. Anti-dsDNA antibodies may be found both in lcSSc and dcSSC [ 46 ], but their clinical significance is unknown.…”

Section: Kidney Involvement In Systemic Sclerosismentioning

confidence: 99%

Kidney Involvement in Systemic Sclerosis

Reggiani

Moroni

Ponticelli³

2022

JPM

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Background: Systemic sclerosis is a chronic multisystem autoimmune disease, characterized by diffuse fibrosis and abnormalities of microcirculation and small arterioles in the skin, joints and visceral organs. Material and Methods: We searched for the relevant articles on systemic sclerosis and kidney involvement in systemic sclerosis in the NIH library of medicine, transplant, rheumatologic and nephrological journals. Results: Half of patients with systemic sclerosis have clinical evidence of kidney involvement. Scleroderma renal crisis represents the most specific and serious renal event associated with this condition. It is characterized by an abrupt onset of moderate to marked hypertension and kidney failure. Early and aggressive treatment is mandatory to prevent irreversible organ damage and death. The advent of ACE-inhibitors revolutionized the management of scleroderma renal crisis. However, the outcomes of this serious complication are still poor, and between 20 to 50% of patients progress to end stage renal disease. Conclusions: Scleroderma renal crisis still represents a serious and life-threatening event. Thus, further studies on its prevention and on new therapeutic strategies should be encouraged.

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Section: Kidney Involvement In Systemic Sclerosismentioning

confidence: 99%

Kidney Involvement in Systemic Sclerosis

Reggiani

Moroni

Ponticelli³

2022

JPM

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“…Patients with SS can have anti-neutrophil cytoplasmic antibodies (ANCAs) [ 165 ] which by themselves can lead to ANCA-associated vasculitis. In patients who have ANCAs, platelets are activated via the thrombin-protease activated receptor (PAR) pathway and produce PEVs [ 162 ].…”

Section: Platelet-derived Extracellular Vesicles In Autoimmunitymentioning

confidence: 99%

The Role of Platelet-Derived Extracellular Vesicles in Immune-Mediated Thrombosis

Eustes

Dayal

2022

IJMS

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Platelet-derived extracellular vesicles (PEVs) play important roles in hemostasis and thrombosis. There are three major types of PEVs described based on their size and characteristics, but newer types may continue to emerge owing to the ongoing improvement in the methodologies and terms used to define various types of EVs. As the literature on EVs is growing, there are continuing attempts to standardize protocols for EV isolation and reach consensus in the field. This review provides information on mechanisms of PEV production, characteristics, cellular interaction, and their pathological role, especially in autoimmune and infectious diseases. We also highlight the mechanisms through which PEVs can activate parent cells in a feedback loop.

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“…Renal biopsy is not required if the presentation is classical of AAV or SRC, thus should only be performed when presentations are clinically ambiguous. 10 There is no specific treatment strategy for SSc-AAV, other than managing the underlying presentation. Treatment has included high-dose steroids, and cyclophosphamide, followed by maintenance therapy with azathioprine or mycophenolate mofetil.…”

Section: Management Strategiesmentioning

confidence: 99%

“…Organ involvement distribution based on autoantibodies in SSc and AAV alone, and in SSc-AAV combined 10. …”

mentioning

confidence: 99%

Review of systemic sclerosis and antineutrophil cytoplasmic antibody vasculitis overlap: Using autoantibodies for a personalised medicine approach

Clark

2022

Journal of Scleroderma and Related Disorders

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Both antineutrophil cytoplasmic antibody-associated vasculitis and systemic sclerosis are rare autoimmune diseases. Both have the potential for significant multi-organ involvement, and both carry high morbidity and mortality. Disease-specific autoantibodies in these conditions allow for risk stratification for organ-based complications, and for personalised therapeutic strategies. The concomitant presentation of antineutrophil cytoplasmic antibody-associated vasculitis and systemic sclerosis is rare, and only reported in up to 1.3% of systemic sclerosis cases. These patients present more frequently with anti-myeloperoxidase and anti-topoisomerase antibody profiles, with increased incidence of interstitial lung disease and renal involvement than would be expected in either disease independently. Appreciating the role of the autoantibodies in each disease state, and where they overlap, allows for the potential of a more personalised approach to managing these complex patients.

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ANCA in systemic sclerosis, when vasculitis overlaps with vasculopathy: a devastating combination of pathologies

Cited by 11 publications

References 47 publications

Kidney Involvement in Systemic Sclerosis

Kidney Involvement in Systemic Sclerosis

The Role of Platelet-Derived Extracellular Vesicles in Immune-Mediated Thrombosis

Review of systemic sclerosis and antineutrophil cytoplasmic antibody vasculitis overlap: Using autoantibodies for a personalised medicine approach

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