Sections

Presentation

History

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

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

Renal manifestations are seen in more than 80% of patients and fall on a spectrum from asymptomatic hematuria to necrotizing crescentic GN causing end-stage kidney disease^[20].

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%)
Testicular pain (2%)
Ocular (1%) - Red eye, ocular pain, decreased visual acuity
Symptoms of sinusitis (1%)

Physical Examination

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.
View Media Gallery
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

Compared to GPA and EGPA, MPA is more closely associated with livedoid changes, and vascular inflammation tends to be deeper in the skin; extravascular granulomas are not present.^[21]

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.

Differential Diagnoses

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Media Gallery

Pulmonary alveolar capillaritis.
Histopathology of alveolar hemorrhage in alveolar capillaritis.
Crescentic glomerulonephritis.
Focal segmental glomerulonephritis.
Histopathology of leukocytoclastic angiitis.
Leukocytoclastic angiitis.

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Author

Mehran Farid-Moayer, MD Adjunct Clinical Faculty, Department of Psychiatry, Sleep Disorders Clinic, Stanford Medical Center

Mehran Farid-Moayer, MD is a member of the following medical societies: American Academy of Sleep Medicine, American Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Spencer T Lowe, MD Rheumatologist, Mills-Peninsula Medical Center, Sutter Health

Spencer T Lowe, MD is a member of the following medical societies: American College of Rheumatology, California Medical Association, International Society for Clinical Densitometry

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Elliot Goldberg, MD Dean of the Western Pennsylvania Clinical Campus, Professor, Department of Medicine, Lewis Katz School of Medicine at Temple University

Elliot Goldberg, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology

Disclosure: Nothing to disclose.

Chief Editor

Vecihi Batuman, MD, FASN Professor of Medicine, Section of Nephrology-Hypertension, Deming Department of Medicine, Tulane University School of Medicine

Vecihi Batuman, MD, FASN is a member of the following medical societies: American College of Physicians, American Society of Hypertension, American Society of Nephrology, Southern Society for Clinical Investigation

Disclosure: Nothing to disclose.

Additional Contributors

Bryan L Martin, DO Associate Dean for Graduate Medical Education, Designated Institutional Official, Associate Medical Director, Director, Allergy Immunology Program, Professor of Medicine and Pediatrics, Ohio State University College of Medicine

Bryan L Martin, DO is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American College of Allergy, Asthma and Immunology, American College of Osteopathic Internists, American College of Physicians, American Medical Association, American Osteopathic Association

Disclosure: Nothing to disclose.

Microscopic Polyangiitis Clinical Presentation

History

Physical Examination

References

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