Microscopic polyangiitis presenting as pulmonary-renal syndrome in a patient with long-standing diffuse cutaneous systemic sclerosis and antibodies to myeloperoxidase

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

“…3 Multiple case reports and case series support this, with most describing SSc patients with normotensive renal failure found to have MPO and pANCA positivity and pauci-immune necrotizing crescentic GN on renal histopathology. 1,3,4,[6][7][8][9][10][13][14][15][16][17][18][19][20][21][22][23][24][25][26] Much of this literature has been reviewed recently. 9,18 Overall, the vast majority of such patients derived benefit from treatment with high-dose corticosteroids and/or IV or oral CYC.…”

“…Another frequently reported vasculitic organ system manifestation, both in our series and the literature, is pulmonary involvement, 1,4,13 with generally poor survival especially in those with pulmonary-renal syndrome. 4,31 In our series, pulmonary involvement was frequent (79%), including alveolar hemorrhage in 5 patients and pulmonary-renal syndrome in 3 patients, #2 with limited SSc and 2 of whom died.…”

“…Hence, it appears that a pulmonary-renal syndrome presentation of microscopic polyangiitis may occur in SSc patients with both limited and diffuse forms of the disease, with poor prognosis. However, in both the patient reported by Wutzl et al, 1 as well as patient #10 in our series, treatment with high dose corticosteroids and cyclophosphamide allowed for survival despite pulmonary-renal syndrome.…”

“…1 Indeed, the most frequent vessel histopathology in scleroderma is subintimal proliferation and periadventitial fibrosis. 2 Although vasculitis is known to occur as a complication of many connective tissue diseases, antimyeloperoxidase (MPO), perinuclear antineutrophil cytoplasmic antibodies (pANCA), and/or clinically evident vasculitis in patients with scleroderma have been reported only rarely.…”

“…Previous case series have suggested a possible association of vasculitis with the limited or CREST variant of scleroderma and features of Sjogren's syndrome, 2 though multiple other case reports and series have described vasculitis in the diffuse variant of scleroderma as well. 1,[3][4][5][6][7][8][9] The clinical significance and prognosis of ANCA-associated vasculitis (AAV) in systemic sclerosis (SSc) is uncertain. Specifically, it is unclear how frequently different vasculitic organ system manifestations occur; whether vasculitis occurs more frequently in patients with limited versus diffuse cutaneous variants of SSc; and what the frequency is of overlap connective tissue disease features including Sjogren's syndrome features and other autoimmune serologic markers in scleroderma patients with vasculitis.…”