Interstitial nephritis without glomerulonephritis in ANCA-associated vasculitis: a case series and literature review

The typical nephrological presentation of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is rapidly progressive glomerulonephritis. AAV-associated interstitial nephritis without apparent glomerular lesions was rare. We reported three local cases of AAV-associated interstitial nephritis without glomerulonephritis confirmed by renal biopsy. Then, a literature search was conducted in PubMed using free text words and MeSH terms related to AAV and interstitial nephritis. Fifteen cases were included, and their demographics, clinical manifestations, laboratory data, renal pathological features, and treatment response were summarized. AAV-associated interstitial nephritis usually affects elderly patients. The common symptoms include fever, arthralgias, and edema. These patients were mostly MPO-ANCA positive. Pathological lesions in the kidney showed diffuse infiltration of inflammatory cells, edema, tubulitis, and fibrosis in the interstitial area. Various immunosuppressive treatments, including glucocorticoids, immunosuppressants, and rituximab, were used, and most of the patients achieved clinical remission. AAV-associated interstitial nephritis is rare but shows a characteristic clinical phenotype, serological results, and pathogenic lesions. Immunosuppressive therapy showed good efficacy in these patients.

Automated summary

The typical nephrological presentation of ANCA-associated vasculitis is rapidly progressive glomerulonephritis, but interstitial nephritis without glomerular lesions is rare. This article reports three cases of AAV-associated interstitial nephritis without glomerulonephritis and summarizes the clinical features, laboratory data, renal pathology, and treatment response of fifteen cases identified through a literature search. AAV-associated interstitial nephritis commonly affects elderly patients and is characterized by fever, arthralgias, and edema. Immunotherapy shows good efficacy in these patients.