Vasculitis in patients with systemic sclerosis and severe digital ischaemia requiring amputation.

Herrick, Ariane L.; Oogarah, P K; Freemont, A. J.; Marcuson, R. W.; Haeney, Mansel; Jayson, M. I. V.

doi:10.1136/ard.53.5.323

Cited by 55 publications

(33 citation statements)

References 9 publications

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“…This may be in part because the subset of SSc patients with normotensive renal failure due to pANCA-positive crescentic GN has only been recognized more recently, since about 1994. Herrick et al 32 reported that 5 out of 9 patients with severe digital ischemia from SSc (all of the limited variant) had histologic evidence of vasculitis. In our current series of 15 cases, we found 6 had skin purpura, 2 had ischemic digital ulcers, 5 had mononeuritis multiplex and/or peripheral neuropathy, and 2 had myopathies.…”

Section: Discussionmentioning

confidence: 99%

ANCA-associated vasculitis in scleroderma: a case series of fourteen patients

Liang

Michet

2011

Rheumatol Rep

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Antimyeloperoxidase (MPO), perinuclear antineutrophil cytoplasmic antibodies (pANCA), and/or clinically evident vasculitis in patients with scleroderma have been reported only rarely. The clinical significance and prognosis of ANCA-associated vasculitis in systemic sclerosis is uncertain. To report a case and identify the clinical characteristics of scleroderma patients with ANCA-associated vasculitis. Patients with both vasculitis and scleroderma occurring between 1976 to 2006 were identified using an electronic diagnostic index. These diagnoses were confirmed by retrospective review of complete medical records. Clinical features and outcomes recorded included age at vasculitis diagnosis, connective tissue disease (CTD) features, type of scleroderma (limited or diffuse); ANCA serology, vasculitic organ system manifestations; and death. Fourteen cases of scleroderma patients with ANCA-associated and/or small vessel vasculitis were identified. The majority (71%) were female, with mean age at vasculitis diagnosis 53 years. Seven patients (50%) had overlap CTD features, and the majority (79%) had limited variant of scleroderma. All of the 10 patients tested were MPO and pANCA positive. Seven patients (50%) had glomerulonephritis, 11 (79%) pulmonary involvement including 3 with pulmonary-renal syndrome, 6 skin purpura, and 5 mononeuritis multiplex and/or peripheral neuropathy. Six patients (43%) died during followup to 2008. The presence of pANCA-associated small vessel vasculitis is a rarely reported complication of scleroderma. It occurs most commonly in women with limited scleroderma and most commonly includes pulmonary and/or renal involvement, including severe organ-threatening manifestations and death. Further studies are needed to clarify the role and clinical impact of ANCA in scleroderma patients with and without vasculitis.

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Section: Discussionmentioning

confidence: 99%

ANCA-associated vasculitis in scleroderma: a case series of fourteen patients

Liang

Michet

2011

Rheumatol Rep

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“…11 A single dose of aspirin usually results, however, in reversal of the ischemic phenomena with resolution of erythromelalgia in the vast majority of patients within 2 to 4 days. 2 Acute digital ischemia may be caused by several entities including collagen vascular diseases, 12 Raynaud's disease, 13 Buerger's disease, 14 peripheral atherosclerosis, 15 heparin-induced thrombocytopenia with thrombosis syndrome, 16 consumption coagulopathy, 17 and many others. Although rare, hematologic disorders, however, have to be considered in the differential diagnosis of acute digital ischemia.…”

Section: Discussionmentioning

confidence: 99%

Digital Ischemia Due to Essential Thrombocythemia: A Case Report

Papadonikolakis

Chloros

Smith

et al. 2007

The Journal of Hand Surgery

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“…Acute digital ischemia may be caused by several entities including collagen vascular diseases [3], Raynaud's disease [4], Buerger's disease [5], peripheral atherosclerosis [6], heparininduced thrombocytopenia with thrombosis syndrome [7], consumption coagulopathy [8] and many others. Although rare, hematologic disorders, however, have to be considered in the differential diagnosis of acute digital ischemia.…”

Section: Discussionmentioning

confidence: 99%

Digital ischemia due to Systemic Sclerosis associated to Essential Thrombocythemia: A case report

Ajili¹,

Mansour²,

Ghedira³

et al. 2013

Our Dermatol Online

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Digital ulcers (DU) are a well-known problem in patients with systemic sclerosis. It is an underestimated complication of the disease causing pain and morbidity. Essential thrombocytosis is another cause of DU. The association of theses two diseases increases the risk of ischemic complications and impairment of hand function which are frequently observed in patients with digital ulcers. This report deals with a 68-year-old patient with rare association of Essential thrombocytosis ,Systemic sclerosis and Raynaud's phenomenon that was refractory to medical treatment of Systemic sclerosis (illoprost,calcium channel blockers) and improved with hydrea R .

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Vasculitis in patients with systemic sclerosis and severe digital ischaemia requiring amputation.

Cited by 55 publications

References 9 publications

ANCA-associated vasculitis in scleroderma: a case series of fourteen patients

ANCA-associated vasculitis in scleroderma: a case series of fourteen patients

Digital Ischemia Due to Essential Thrombocythemia: A Case Report

Digital ischemia due to Systemic Sclerosis associated to Essential Thrombocythemia: A case report

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