Systemic lupus erythematosus and antineutrophil cytoplasmic antibody-associated vasculitis overlap syndrome in a 77-year-old man: A case report

Abstract

BACKGROUND Systemic lupus erythematosus (SLE) and antineutrophil cytoplasmic antibody-associated vasculitis (AAV) are classically thought to cause renal impairment and small vessel vasculitis with different pathophysiologies. Their overlap constitutes a rare rheumatologic disease. To date, only dozens of such cases with biopsy-proven glomerulonephritis have been reported worldwide typically in women of childbearing age. Here, we present a unique clinical case due to its rarity and individualized treatment of a Chinese man in his eighth decade of life. CASE SUMMARY A 77-year-old man was admitted to several hospitals for shortness of breath and received nonspecific treatments over the past 3 years. As his symptoms were not completely relieved, he visited our hospital for further treatment. Laboratory examinations revealed kidney dysfunction, severe anaemia, hypocom-plementemia, glomerular proteinuria, and microscopic haematuria. Antinuclear antibodies, as well as anti-dsDNA antibodies, were positive. Computed tomography of the chest showed right pleural effusion. Renal biopsy was performed, and histology suggested crescentic glomerulonephritis, pauci-immune type. After treatment with plasmapheresis, glucocorticoid, and cyclo-phosphamide, the disease was in remission, and the patient remained in a stable condition for over 3 years post-hospital discharge. CONCLUSION Due to its complexity and rarity, SLE and AAV overlap syndrome is easily misdiagnosed. An accurate diagnosis and treatment at the earliest stage may significantly improve the condition and reduce irreversible organ injury.

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

“…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 ].…”

“…Rituximab and cyclophosphamide are both used for induction therapy for AAV though authors and physicians tend to favor rituximab over cyclophosphamide as it is found to be less toxic and tolerated well by patients [ 18 ]. Further treatment with plasma exchange can be utilized to rapidly remove pathogenic autoantibodies and prevent further deterioration of the kidneys [ 16 ].…”