Microscopic polyangiitis natural history, complications and prognosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1], Ali Poyan Mehr, M.D. [2]; Associate Editor(s)-in-Chief: Vamsikrishna Gunnam M.B.B.S [3], Krzysztof Wierzbicki M.D. [4]

Overview

If left untreated, Microscopic polyangiitis can progress to end stage renal failure and respiratory failure. Complications of Microscopic polyangiitis include, alveolar hemorrhage, end stage renal failure, osteoarticular disease, and infections. The prognosis of Microscopic polyangiitis.

Natural History

If left untreated, Microscopic polyangiitis can progress to end stage renal disease and respiratory failure.

Complications

Possible complications of Microscopic polyangiitis include:

Abdominal sepsis
Meningoencephalititis
Alveolar hemorrhage
Osteoarticular disease
End stage renal failure

Prognosis

In the past the prognosis of Microscopic polyangiitis was fatal. Patients with Microscopic polyangiitis that was untreated had a mortality rate of about 90% in 2 years. With the advent of medications such as glucocorticoids and cyclophosphamide the mortality rate has decreased to a rate of 12 to 44 percent in about 4 to 10 years.^[1]

The following are favorable prognostic factors:

Aggressive treatment with immunosuppressants together with a corticosteriod.
Renal-pulmonary syndrome is not present.

The following are poor prognostic factors:

Five-Factor Score Assessment
Proteinuria > 1g/d
Creatinine > 140mm/l
Cardiomyopathy
Severe GI manifestations
CNS involvement

According to the Five-Factor Score, a score of 0, 1, or greater than 2 has the following mortality rate:

Five-Factor Score
Score	Mortality Rate
0	12%
1	26%
>2	46%

It has also been concluded that age greater than 65 is also a risk factor for mortality.^[4]

MPO‐ANCAs were a marker of poor survival and increased mortality rate in this population of patients with AAV.^[2]

References

↑ Corral-Gudino L, Borao-Cengotita-Bengoa M, Del Pino-Montes J, Lerma-Márquez JL (2011). “Overall survival, renal survival and relapse in patients with microscopic polyangiitis: a systematic review of current evidence”. Rheumatology (Oxford). 50 (8): 1414–23. doi:10.1093/rheumatology/ker112. PMID 21406467.
↑ Berti A, Cornec D, Crowson CS, Specks U, Matteson EL (December 2017). “The Epidemiology of Antineutrophil Cytoplasmic Autoantibody-Associated Vasculitis in Olmsted County, Minnesota: A Twenty-Year US Population-Based Study”. Arthritis Rheumatol. 69 (12): 2338–2350. doi:10.1002/art.40313. PMID 28881446.

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[pmid21406467-1] Corral-Gudino L, Borao-Cengotita-Bengoa M, Del Pino-Montes J, Lerma-Márquez JL (2011). “Overall survival, renal survival and relapse in patients with microscopic polyangiitis: a systematic review of current evidence”. Rheumatology (Oxford). 50 (8): 1414–23. doi:10.1093/rheumatology/ker112. PMID 21406467.

[pmid288814463-2] Berti A, Cornec D, Crowson CS, Specks U, Matteson EL (December 2017). “The Epidemiology of Antineutrophil Cytoplasmic Autoantibody-Associated Vasculitis in Olmsted County, Minnesota: A Twenty-Year US Population-Based Study”. Arthritis Rheumatol. 69 (12): 2338–2350. doi:10.1002/art.40313. PMID 28881446.

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