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Spontaneous and protracted partial remission of microscopic polyangiitis
Martin Plaisance1, David J A Goldsmith
1Nephrology and Transplantation, Guy's and St-Thomas' NHS Trust, Guy's Hospital, London, United Kingdom.
Abstract:
Microscopic polyangiitis (MPA), or microscopic polyarteritis, is an idiopathic small vessel vasculitis that frequently causes glomerular damage and renal failure and skin and lung damage in many cases. The renal lesions include focal necrotizing glomerulonephritis, extracapillary proliferative (crescentic) glomerulonephritis, and tubulointerstitial infiltration with polymorphonuclear leukocytes and lymphocytes. MPA often is associated with the presence of antineutrophil cytoplasmic autoantibody (ANCA) (myeloperoxidase positive) as a diagnostic marker. MPA commonly is regarded as a serious condition that places the survival of the kidneys and the patient at risk. Typically, there is a prodrome of some weeks to months, with rapid decline in renal function and dialysis as a potential outcome if intensive immunosuppressive treatment is not given or is delayed. We describe an otherwise typical case of MPA occurring in a 52-year-old woman presenting with multisystem disease, antimyeloperoxidase ANCA antibodies, renal impairment, and necrotizing crescentic glomerulonephritis in whom this usual sequence of events was not followed because the patient refused steadfastly to have any treatment for nearly a decade. Renal function remained stable for nearly 10 years, although there were persistent proteinuria, microscopic hematuria, and antimyeloperoxidase ANCA antibodies. A late renal-pulmonary relapse occurred, and immunosuppression was permitted only briefly. Prolonged renal and patient survival in the absence of immunosuppressive treatment has been reported rarely in this context.
Insights
Microscopic polyangiitis (MPA) is a serious vasculitis. A rare case shows prolonged survival without treatment, defying typical rapid kidney decline and highlighting treatment refusal
Area of Science:
- Nephrology
- Rheumatology
- Immunology
Background:
- Microscopic polyangiitis (MPA) is an idiopathic small vessel vasculitis often leading to kidney damage and failure.
- MPA is characterized by necrotizing glomerulonephritis and is frequently associated with myeloperoxidase-antineutrophil cytoplasmic autoantibodies (MPO-ANCA).
- The condition typically requires prompt immunosuppressive treatment to prevent rapid renal function decline and potential dialysis.
Observation:
- A 52-year-old woman with MPA, MPO-ANCA positivity, and renal impairment refused treatment for nearly a decade.
- Despite refusing treatment, her renal function remained stable with persistent proteinuria and hematuria.
- A late renal-pulmonary relapse occurred, necessitating brief immunosuppression.
Findings:
- This case challenges the typical aggressive progression of MPA, demonstrating prolonged renal stability without immunosuppression.
- Persistent MPO-ANCA positivity and sub-nephrotic proteinuria were noted despite stable renal function.
- The patient experienced a late-onset renal-pulmonary relapse, indicating disease activity despite the prolonged quiescent period.
Implications:
- This rare case suggests that some MPA patients may have a more indolent disease course than typically observed.
- It underscores the variability in MPA presentation and progression, even in the presence of diagnostic biomarkers.
- Further research is needed to understand factors influencing MPA's clinical course and the potential for long-term remission without immunosuppression.