Microscopic polyangiitis natural history, complications and prognosis

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

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.

The following are favorable prognostic factors:

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%

^[3] It has also been concluded that age greater than 65 is also a risk factor for mortality.^[4]