Microscopic Polyangiitis Clinical Presentation

Updated: Jul 31, 2018
  • Author: Mehran Farid-Moayer, MD; Chief Editor: Vecihi Batuman, MD, FASN  more...
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Presentation

History

Constitutional manifestations of microscopic polyangiitis (MPA) include the following:

  • Fever (55%)
  • Malaise, fatigue, flulike syndrome
  • Myalgia (48%)
  • Weight loss (72%)

Other manifestations of MPA include the following:

  • Skin - Rash (50%)
  • Pulmonary - Hemoptysis (11%), dyspnea, cough
  • Cardiovascular – Chest pain, symptoms of heart failure
  • Gastrointestinal (GI) - GI bleeding, abdominal pain
  • Neurologic - Peripheral nervous system involvement manifesting as mononeuritis multiplex (57%); CNS involvement manifesting as seizures (11%)
  • Arthralgias (10-50%)
  • Myalgias (40%)
  • Testicular pain (2%)
  • Ocular (1%) - Red eye, ocular pain, decreased visual acuity
  • Symptoms of sinusitis (1%)
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Physical

The physical examination findings include fever and the manifestations of specific organ system involvement. A dermato-pulmonary-renal syndrome is the feature of the disease.

Skin findings are as follows:

  • Leukocytoclastic angiitis and its palpable purpura  - Leukocytoclastic purpura could be a manifestation of the systemic vasculitides or could be a stand-alone skin disorder (see image below.)

    Leukocytoclastic angiitis. Leukocytoclastic angiitis.
  • Palpable purpura (41%)

  • Livedo reticularis (12%)

  • Skin ulcerations

  • Necrosis and gangrene

  • Necrotizing nodules

  • Digital ischemia (7%)

  • Urticaria - Vasculitis-associated urticaria that lasts longer than 24 hours

Respiratory findings in the lower respiratory system include pulmonary rales and respiratory distress. In the upper respiratory tract, sinusitis is less frequent than in granulomatosis with polyangiitis (Wegener granulomatosis).

Cardiovascular findings include the following:

  • Hypertension (34%)

  • Signs of heart failure (17%)

  • Myocardial infarction (2%)

  • Pericarditis (10%)

Gastrointestinal findings include the following:

  • Gastrointestinal bleeding

  • Bowel ischemia and perforation

  • Pancreatitis

Ocular findings (1%) include the following:

  • Retinal hemorrhage

  • Scleritis

  • Uveitis

Renal findings comprise signs of uremia in advanced renal failure; 8% of patients with MPA present with renal failure and require hemodialysis. 

Musculoskeletal findings include synovitis and arthritis.

Neurologic findings are as follows:

  • Mononeuritis multiplex - Most frequent neurologic manifestation of the disease (57%)

  • CNS involvement - Includes meningeal vasculitis (11%)

Orchitis is present in 2% of male patients.

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