A unique case of anti-GBM disease with concomitant anti-PLA2R positivity

Abstract

Background Concomitant occurrence of anti-GBM disease and anti-PLA2R positive membranous nephropathy have been previously described. However, to the best of our knowledge, this is the first case report that documents the co-occurrence of the diseases proven by both serologic and histologic methods. Case presentation A 51-year-old woman presented to hospital with nausea, bilateral lower extremity edema, dyspnea, dark urine, and then anuria. Symptoms developed one month after an upper respiratory tract infection. Laboratory results showed acute kidney injury, and hypoalbuminemia. Immunologic examination revealed both anti-GBM and anti-PLA2R positivity. Kidney biopsy demonstrated the histological features of Goodpasture’s disease and anti-PLA2R positive membranous nephropathy. Steroid, cyclophosphamide, and plasmapheresis were commenced. Despite the combined immunosuppressive, the patient remained on renal replacement therapy. Conclusions Microbial kidney injury can trigger multiple autoimmune diseases. The simultaneous occurrence of anti-glomerular basement (anti-GBM) disease and membranous nephropathy is extremely rare. Delayed recognition leads to delayed treatment, causing worse renal and patient outcomes, as well as increased financial costs.

Idiopathic membranous nephropathy and synchronous mononeuritis multiplex secondary to idiopathic small vessel vasculitis

“…De novo vasculitis following the diagnosis of membranous nephropathy has been reported; however, cases are renal limited, typically present as crescentic glomerulonephritis, and are associated with de novo ANCA or anti-GBM antibody positivity 4 13–16. This is the first case report to document the synchronous occurrence of mononeuritis multiplex secondary to small vessel vasculitis and idiopathic membranous nephropathy, and the subsequent achievement of complete remission with rituximab.…”

“…A postulated mechanism for systemic immunological disorders following membranous nephropathy is the exposure of glomerular podocyte, endothelial and basement membrane proteins to immune system detection and subsequent attack 15 16. It is unclear whether the association documented in this case report ought to be interpreted through Occam’s razor; a new immunological syndrome or by Hickam’s dictum; two unrelated idiopathic immunological disorders occurring simultaneously.…”

Emerging molecular mechanisms and therapeutics of autoimmune liver or kidney diseases

“…Many polyphenols, such as curcumin and resveratrol, have been reported to be potent inhibitors of NF-kB, inhibiting NF-kB translocation to the nucleus, inhibiting its binding from targeting DNA and subsequent transcription of pro-inflammatory cytokines, inhibiting phosphorylation or ubiquitination of signaling molecules, and inhibiting degradation of IkB (99). Polyphenols also target TLR4, the upstream of NF-kB, mitigating the inflammatory response through the TLR4/NF-kB signaling pathway (100). Resveratrol can suppress excessive inflammatory responses by interfering with HMGB1-mediated activation of the TLR4/NF-kB signaling pathway (101).…”

“…Indeed, despite delaying transplantation and implementing standardized immunosuppressive regimens, sporadic cases of recurrence still occur (98,99). Furthermore, disease recurrence has been observed even in the absence of serum anti-GBM antibodies (100,101). The occurrence of such cases may be attributed to the presence of specific subtypes of anti-GBM antibodies that are not detectable by the tests currently employed (102,103).…”

NETosis: an emerging therapeutic target in renal diseases

“…Anti-glomerular basement membrane (GBM) disease is a rare autoimmune disorder in which antibodies are produced against type IV collagen and presents with rapidly progressive glomerulonephritis and alveolar hemorrhage (100). Anti-GBM IgG binds FcgRIIa in a shear-force-dependent manner.…”