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

Abstract

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.

ANCA-Associated Vasculitis in Patient with CREST-Syndrome - Case Report

“…In our unit database for 1994-2015 years we found 87 patient with AAV and 7 patients with SS, the only one with AAV and SS association is presented here. Our case data are in agreement with recent observations [7,9], suggesting that presence of ANCA-associated vasculitis with MPO-ANCA is a rarely reported complication of scleroderma, occurring most commonly in women with limited or CREST variant of scleroderma, and constituting a differential diagnostic challenge with a scleroderma renal crisis, especially in unusual normotensive presentations. Same authors indicate that immunosuppressive therapy is effective, but with significant mortality or progression to ESRD.…”

“…The presence of ANCA in patients with SS was first described in 1996 [4] and since that has been reported to be rare and not presenting clinical significance. However since 1996 to 2006 about 50 cases with association of SS and AAV were described, and more recently additional cases were reported, and the clinical features of such association were recognized [5][6][7][8][9][10].…”

Proteinase 3 (PR3)-antineutrophil cytoplasmic antibody (ANCA)-associated vasculitic neuropathy in diffuse cutaneous systemic sclerosis: a rare duo

“…and most commonly reported organ involvement is glomerulonephritis (often renal limited vasculitis). While vasculitic peripheral neuropathy has been rarely reported,8–10 in conjunction with other vasculitic manifestations in SSc-AAV, an isolated vasculitic neuropathy as a manifestation of SSc-AAV overlap is exceedingly uncommon. The large majority of SSc-AAV overlap cases reported in the literature are MPO-p-ANCA positive and presence of PR3-c-ANCA is very uncommon (table 1).…”

“…The overlap of SSc-AAV is reported more commonly among those with lcSSc (79%–88%),8 10 those with Scl-70 antibody (59%–77%) 3 11. and most commonly reported organ involvement is glomerulonephritis (often renal limited vasculitis).…”

Prevalence and clinical association of the presence of anti-neutrophilic cytoplasmic antibody in systemic sclerosis

“…The prevalence of ANCA in SSc is reported to be between 2.4% and 11.7% [11][12][13][14][15]. ANCA is commonly revealed in lcSSc or CREST syndrome [7]. The most common manifestation in SSc was renal involvement, particularly crescentic glomerulonephritis [7].…”