Microscopic Polyangiitis Workup

  • Author: Mehran Farid-Moayer, MD; Chief Editor: Vecihi Batuman, MD, FACP, FASN   more...
 
Updated: Jan 24, 2012
 

Laboratory Studies

  • Hematology laboratory studies
    • Leukocytosis
    • Anemia (normocytic anemia)
  • Elevated erythrocyte sedimentation rate (ESR)
  • Renal tests
    • Elevated serum BUN and creatinine (70%)
    • Abnormal urine sediment
    • Proteinuria (80%)
    • Hematuria (67%)
    • Leukocyturia (44%)
    • Erythrocyte casts
  • Antineutrophil cytoplasmic antibodies
    • ANCA positive (80%)
    • Perinuclear ANCA related to myeloperoxidase ANCA (60%)
    • Cytoplasmic ANCA related to proteinase-3 ANCA (40%)
  • Blood cultures to rule out bacterial endocarditis
  • Normal C3 and C4
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Imaging Studies

  • Chest radiograph
    • Bilateral irregular, nodular, and patchy opacities
    • Pulmonary cavitary lesions (less frequently than Wegener granulomatosis)
    • Diffuse parenchymal infiltrates secondary to pulmonary alveolar capillaritis and hemorrhage (see image below) Pulmonary alveolar capillaritis. Pulmonary alveolar capillaritis.
  • Other imaging studies – Indicated for the complications of the disease and specific organ system involvement, such as abdominal CT scan for pancreatitis or mesenteric angiography to differentiate from polyarteritis nodosa
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Other Tests

  • Ordered according to the specific organ system involved
    • ECG indicated for myocardial infarction, pericarditis, and congestive heart failure
    • Gastrointestinal endoscopy in case of gastrointestinal bleeding
    • Electromyography (EMG) in case of clinical evidence of neuropathy
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Procedures

  • Skin biopsy if skin is involved
  • Open lung biopsy
  • Renal biopsy to help diagnose crescentic glomerulonephritis
  • Sural nerve biopsy if EMG results are consistent with sural nerve involvement
  • The results of a retrospective study suggest muscle biopsy may be helpful for the diagnosis of systemic vasculitides even in the absence of myalgias or CPK elevation.[7]
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Histologic Findings

Pathologically, microscopic polyangiitis (MPA) may cause necrotizing arteritis that is histologically identical to that caused by polyarteritis nodosa.

According to the Chapel Hill consensus conference on the classification of small vessel vasculitis, polyarteritis nodosa and MPA are distinguished pathologically by the absence of vasculitis in vessels other than arteries in patients with polyarteritis nodosa and the presence of vasculitis in vessels smaller than arteries, such as arterioles, venules, and capillaries, in patients with MPA.

Because of sparing of muscular and larger vessels in MPA, macroscopic infarcts similar to those seen in polyarteritis nodosa are uncommon. Histologically, segmental fibrinoid necrosis of the media may be present, but in some, the change is limited to infiltration with neutrophils, which become fragmented as they follow the vessel wall (leukocytoclasia). The term leukocytoclastic angiitis is given to such lesions, most commonly found in postcapillary venules.

Immunoglobulins and complement components may be present in the vascular lesions of the skin. The paucity of immunoglobulin is demonstrable using immunofluorescence microscopy (ie, pauci-immune injury).

The glomerulonephritis in MPA is characterized by focal necrosis, crescent formation, and the absence or paucity of immunoglobulin deposits. Pulmonary manifestation is in the form of pulmonary alveolar capillaritis. Biopsy of the muscle and sural nerve may reveal necrotizing vasculitis in small and medium vessels.

Histopathology of alveolar hemorrhage in alveolar Histopathology of alveolar hemorrhage in alveolar capillaritis. Crescentic glomerulonephritis. Crescentic glomerulonephritis. Focal segmental glomerulonephritis. Focal segmental glomerulonephritis. Histopathology of leukocytoclastic angiitis. Histopathology of leukocytoclastic angiitis.
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Contributor Information and Disclosures
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, and American Thoracic Society

Disclosure: Nothing to disclose.

Coauthor(s)

Spencer T Lowe, MD  Rheumatologist, Private Practice, Peninsula Medical Group, Burlingame, CA

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

Disclosure: Nothing to disclose.

Specialty Editor Board

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, and American Osteopathic Association

Disclosure: Nothing to disclose.

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

Disclosure: Medscape Salary Employment

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

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

Disclosure: Nothing to disclose.

Alex J Mechaber, MD, FACP  Senior Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine

Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine

Disclosure: Nothing to disclose.

Chief Editor

Vecihi Batuman, MD, FACP, FASN  Professor of Medicine, Section of Nephrology-Hypertension, Tulane University School of Medicine; Chief, Medicine Service, Southeast Louisiana Veterans Health Care System

Vecihi Batuman, MD, FACP, FASN is a member of the following medical societies: American College of Physicians, American Society of Hypertension, American Society of Nephrology, and International Society of Nephrology

Disclosure: Nothing to disclose.

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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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