Microscopic Polyangiitis


(aka Microscopic Polyarteritis, Polyangiitis Overlap Syndrome, Systemic Necrotizing Vasculitis, Allergic Vasculitis)

Epidemiology


Physiology and Pathology


Diagnosis


Clinical Manifestations

(multi-system involvement with symptoms usually present for only days-weeks before presentation)

Renal Manifestations

(absence of HTN aids in differentiation from Polyarteritis Nodosa)

Constitutional Manifestations (common)

(may precede onset of renal disease by months)

Derm Manifestations (35-60% of cases)

Neuro Manifestations (60% of cases)

Pulmonary Manifestations (10-30% of cases)

(usually severe and may be life-threatening)
(abence of asthma aids in the differentiation from Churg-Struass Syndrome)

Upper Airway Manifestations (5-30% of cases)

Rheum Manifestations

GI Manifestations (35-55% of cases)

(relative sparing of abdominal viscera)

Ocular Manifestations (0-30% of cases)

Cardiac Manifestations (10-15% of cases)


Prognosis

Factors Associated with Poor Survival


Treatment

European Vasculitis Study Group Recommendations for Induction Therapy

(induction therapy is typically given until remission has been achieved or for the first 1 year)

Maintenance Therapy

Side Effects of Therapy

Relapse


References