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

Abstract

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.

Salivary Alterations of Myeloperoxidase in Patients with Systemic Diseases: A Systematic Review

“…The authors suggest that MPO as a neutrophil intracellular protein indicates that repeated neutrophil destruction caused by altered autoimmunity could be involved in the pathogenesis of SS. In patients with ANCA-associated vasculitis in primary SS course, most cases present anti-MPO specificity [81,82].…”

A case of Sjögren syndrome and anti-neutrophil cytoplasmic antibody-associated vasculitis

“…The co-occurrence of these conditions was first reported in 1992 by Böttinger et al (3). Since then, nearly 37 cases of SS and AAV have been reported in the literature (4)(5)(6)(7)(8)(9)(10)(11)(12). Kidney involvement has been observed in 2-67% of patients with SS, in 18% of those with AAV at presentation, and in 64% (24 of the 37 patients) of patients with co-occurrence of SS and AAV (2,(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20).…”

“…Since then, nearly 37 cases of SS and AAV have been reported in the literature (4)(5)(6)(7)(8)(9)(10)(11)(12). Kidney involvement has been observed in 2-67% of patients with SS, in 18% of those with AAV at presentation, and in 64% (24 of the 37 patients) of patients with co-occurrence of SS and AAV (2,(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20). In these predominantly older and female patients, the emergence of AAV was usually documented approximately 5-15 years later than the diagnosis of SS (4,14).…”