A Case of Systemic Lupus Erythematosus and Antineutrophil Cytoplasmic Antibodies-Associated Vasculitis Overlap Syndrome

Abstract

An overlap of systemic lupus erythematosus (SLE) and antineutrophil cytoplasmic antibodies- (ANCA-) associated vasculitis (AAV) is extremely rare: approximately 40 cases have been reported to date. A literature review indicates that they are more common in women in their forties, and simultaneous onset has been reported in 69% of cases. In addition, both lupus nephritis and ANCA-associated glomerulonephritis were observed on renal biopsy. This report presents the case of a 35-year-old woman with an 8-month history of polyarthralgia who was admitted to our hospital. She was diagnosed with SLE due to typical clinical presentation of the disease: polyarthritis, lymphocytopenia, hypocomplementemia, presence of antinuclear and anti-dsDNA antibodies, and proteinuria. However, purpura were scattered, and the titer of antimyeloperoxidase-antineutrophil cytoplasmic antibodies (MPO-ANCA) was high. A skin biopsy revealed leukocytoclastic vasculitis that involved poor immune complex deposition. A renal biopsy showed necrotizing glomerulonephritis with cellular and fibrocellular crescent formation that involved deposition of IgM and C3c only in the mesangial area and the peripheral capillaries. Additionally, no electron-dense deposits were observed under electron microscopy. These pathological findings were consistent with AAV rather than with SLE. Therefore, we finally diagnosed the patient with both SLE and microscopic polyangiitis. After treatment with methylprednisolone and intravenous cyclophosphamide pulse therapies, renal function improved and MPO-ANCA levels decreased. In cases of suspected overlap between SLE and AAV, appropriate diagnosis and treatment are important.

Diagnosing and Managing Clinically Silent Lupus Nephritis and Anti-neutrophil Cytoplasmic Antibody-Associated Vasculitis Overlap Syndrome: A Clinical Challange

“…Usually, LN develops within the first three years following the diagnosis of SLE [ 9 ]. Testing for anti-dsDNA, anti-C1q autoantibodies, and complement levels (C3 and C4) should be considered in patients with suspected LN [ 7 ]. Levels of anti-ds DNA Ab reflect disease presence and activity in patients with LN [ 10 ].…”

“…It is observed that the overlap of LN and AAV is mostly present in women of child-bearing age who present with severe clinical presentation, including severe renal impairment. Renal biopsy tends to reveal a mixed involvement of immune complex-mediated renal injury, as seen in LN, and evidence of necrotizing and crescentic glomerulonephritis, as seen in AAV [ 7 , 16 ].…”

“…A literature review indicates around 40 cases of LN overlapped with AAV. P-ANCA and MPO-ANCA were found to be positive in 95% and 82% of cases, respectively [ 7 ]. Herein, we describe an unusual presentation of acute renal failure (ARF) with serologic and histopathologic features suggestive of both LN and AAV overlap syndrome.…”

Subarachnoid Hemorrhage in Autoimmune Vasculitis: A Rare Presentation of Systemic Lupus Erythematosus-Antineutrophil Cytoplasmic Autoantibody-Associated Vasculitis Overlap Syndrome

“…Overlap syndrome is a connective tissue disorder that meets the criteria for at least two known autoimmune diseases [ 4 ]. The concomitant presentation of both SLE and AAV as overlap syndrome is rare, with only a few cases have been reported in the past [ 5 ]. Here, we present a rare case of subarachnoid hemorrhage as an initial presentation of SLE-AAV overlap syndrome.…”