Pediatric Polyarteritis Nodosa Workup

  • Author: Akaluck Thatayatikom, MD; Chief Editor: Lawrence K Jung, MD   more...
 
Updated: Nov 16, 2011
 

Laboratory Studies

To avoid delay in diagnosis of polyarteritis nodosa (PAN), consider vasculitis when a patient presents with multisystem involvement with persistent nonspecific signs and symptoms. Nonspecific inflammatory changes commonly observed in individuals with PAN include the following:

  • Complete blood count (CBC) - This may reveal normochromic anemia, polymorphonuclear (PMN) leukocytosis, and thrombocytosis; although it occurs in PAN, eosinophilia greater than 1.5 × 109/L commonly (14%) accompanies pulmonary involvement in persons with microscopic polyangiitis (MPA), predominantly in Churg-Strauss syndrome (allergic angiitis and granulomatosis)
  • Erythrocyte sedimentation rate (ESR) - This is elevated (usually above 60 mm/h) and follows disease activity
  • C-reactive protein (CRP) level - Increased
  • Antivisceral epithelial cell antibody - Possibly present
  • Serum albumin (a reverse acute-phase reactant) level - Possibly decreased

Organ involvement is assessed by means of the following determinations:

  • Creatinine concentration
  • Urinalysis
  • 24-hour urine collection - Increased protein loss is more characteristic of MPA than of classic PAN
  • Creatine kinase level

Tests for the following are also performed to differentiate PAN from other conditions:

  • Antineutrophil cytoplasmic antibody (ANCA) - About 20% of patients with classic PAN are positive for perinuclear ANCA (P-ANCA); in patients with microscopic polyangiitis (MPA), 40% are positive for cytoplasmic ANCA (C-ANCA), 50% are positive for P-ANCA, and 10% are negative for ANCA; in persons with Wegener granulomatosis, 75% are positive for C-ANCA, 20% are positive for P-ANCA, and 5% are negative for ANCA
  • Hepatitis B surface antigen (HBsAg), immunoglobulin (Ig) M anti–HB core, anti–hepatitis C virus (HCV) - HBsAg is present in 10-45% of patients with PAN, but results are mostly negative in persons with MPA
  • Blood cultures
  • Antinuclear antibody (ANA) - ANA is present in low titers in individuals with PAN (as many as one third of individuals with MPA)
  • Cryoglobulins - These are associated with rheumatoid factor and hypocomplementemia
  • Serum complement - C3 or C4 levels are depressed in 25% of patients with PAN but may be elevated or within reference range in patients with MPA
  • Rheumatoid factor - This is usually present in low titers in individuals with PAN but may be found in 40-50% of patients with MPA
  • Antiphospholipid antibodies
  • Antiglomerular basement antibodies (differential for Goodpasture syndrome)
  • Angiotensin-converting enzyme (differential for sarcoidosis)
  • Low-titer circulating immune complexes
  • Increased platelet activating factor levels
  • Increased factor VIII-related antigen (von Willebrand factor antigen)
Next

Radiography and Angiography

Chest radiography reveals interstitial infiltrates in patients with hypoxemia and respiratory distress. Sinus radiography may be obtained to exclude a granulomatous vasculitis (eg, Wegener granulomatosis).

Consider arteriography when involved organs are not accessible. Angiographic abnormalities include the following:

  • Long segments of smooth arterial stenosis alternating with areas of normal or dilated arteries (fusiform or saccular aneurysm)
  • Smooth tapered occlusions
  • Thrombosis
  • Absence or nondominance of arterial plaques, irregularities, and ulcerations
  • Multiple intraparenchymal microaneurysms - These are considered pathognomonic; 60% of patients with clinical PAN have aneurysms, and in most cases, more than 10 aneurysms are present
Previous
Next

Other Tests

Doppler ultrasonography may reveal arterial narrowing and dilatations.

Electrocardiography (ECG) may be indicated for assessment of cardiac manifestations.

Electromyography (EMG) or nerve conduction studies may be helpful.

A single biopsy procedure followed by angiography is calculated to have 85% sensitivity and 96% specificity for diagnosis of PAN.

Previous
Next

Histologic Findings

Histopathologic examination demonstrates transmural inflammation of the arterial wall with a heavy infiltrate of polymorphs, eosinophils, and mononuclear cells. This inflammation frequently is accompanied by fibrinoid necrosis of the inner half of the vessel wall. Thrombus can be observed (see the images below).

Histopathology of kidney of individual with polyarHistopathology of kidney of individual with polyarteritis nodosa demonstrates transmural inflammation of arterial wall with heavy infiltrate of polymorphonuclear cells, eosinophils, and mononuclear cells, along with fibrinoid necrosis of inner half of vessel wall. Histopathology of lung of individual with polyarteHistopathology of lung of individual with polyarteritis nodosa demonstrates arterial wall with heavy infiltrate of polymorphonuclear cells, eosinophils, and mononuclear cells and fibrinoid necrosis of vessel wall. Histopathology of kidney of individual with polyarHistopathology of kidney of individual with polyarteritis nodosa demonstrates transmural inflammation of arterial wall with heavy infiltrate of polymorphonuclear cells, eosinophils, and mononuclear cells; fibrinoid necrosis of inner half of vessel wall; and thrombus.

The lesion can be segmental, with normal arterial walls interspersed with diseased areas; it has a predilection for bifurcations. The acute inflammatory infiltrate disappears at a later stage and is replaced by fibrous thickening of the vessel wall. Later, only marked fibrotic thickening is noted.

The presence of all stages of activity in different vessels or within the same vessel at a particular time is a characteristic finding in PAN.

Previous
Proceed to Treatment & Management
 
 
Contributor Information and Disclosures
Author

Akaluck Thatayatikom, MD  Associate Professor and Chief, Department of Pediatrics, Division of Pediatric Allergy, Immunology, and Rheumatology, University of Kentucky College of Medicine

Akaluck Thatayatikom, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American College of Rheumatology, Childhood Arthritis and Rheumatology Research Alliance, and Clinical Immunology Society

Disclosure: Nothing to disclose.

Chief Editor

Lawrence K Jung, MD  Chief, Division of Pediatric Rheumatology, Children's National Medical Center

Lawrence K Jung, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Rheumatology, Clinical Immunology Society, and New York Academy of Sciences

Disclosure: Nothing to disclose.

Additional Contributors

Christine B Bernal, MD Clinical Faculty, Faculty of Medicine and Surgery, University of Santo Tomas, Philippines; Head, Section of Pediatric Rheumatology, University of Santo Tomas Hospital, Philippines

Christine B Bernal, MD is a member of the following medical societies: Philippine Medical Assocation

Disclosure: Nothing to disclose.

James M Oleske, MD, MPH François-Xavier Bagnoud Professor of Pediatrics, Director, Division of Pulmonary, Allergy, Immunology and Infectious Diseases, Department of Pediatrics, New Jersey Medical School

James M Oleske, MD, MPH is a member of the following medical societies: Academy of Medicine of New Jersey, American Academy of Pediatrics, American Public Health Association, American Society for Microbiology, Infectious Diseases Society of America, and Pediatric Infectious Diseases Society

Disclosure: Nothing to disclose.

Donald A Person, MD Expert Consultant in Pediatrics, Pediatric Rheumatology, and Telemedicine, Tripler Army Medical Center; Emeritus Professor of Pediatrics, F Edward Herbert School of Medicine, Uniformed Services University of the Health Sciences; Retired Clinical Professor of Pediatrics and Public Health, University of Hawaii, John A Burns School of Medicine

Donald A Person, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Rheumatology, American Medical Association, American Pediatric Society, American Society for Microbiology, American Society of Tropical Medicine and Hygiene, Association of Military Surgeons of the US, Clinical Immunology Society, Federation of American Societies for Experimental Biology, Pediatric Infectious Diseases Society, Society for Experimental Biology and Medicine, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Muthukumar Vellaichamy, MD, FAAP Clinical Assistant Professor, Department of Pediatrics, University of Kansas School of Medicine-Wichita, Wesley Medical Center

Muthukumar Vellaichamy, MD, FAAP is a member of the following medical societies: American Academy of Pediatrics and Society of Critical Care Medicine

Disclosure: Nothing to disclose.

Mary L Windle, PharmD, Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

References

  1. [Guideline] Newburger JW, Takahashi M, Gerber MA, et al. Diagnosis, treatment, and long-term management of Kawasaki disease: a statement for health professionals from the Committee on Rheumatic Fever, Endocarditis and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association. Circulation. Oct 26 2004;110(17):2747-71. [Medline].

  2. Meeuwissen J, Maertens J, Verbeken E, Blockmans D. Case reports: testicular pain as a manifestation of polyarteritis nodosa. Clin Rheumatol. Nov 2008;27(11):1463-6. [Medline].

  3. Richard T, Delree P, Fumiere E, Vanhaeverbeek M. [Polyarteritis nodosa limited to the right leg]. Rev Med Brux. May-Jun 2008;29(3):206-7. [Medline].

  4. Nakamura T, Kanazawa N, Ikeda T, et al. Cutaneous polyarteritis nodosa: revisiting its definition and diagnostic criteria. Arch Dermatol Res. Jan 2009;301(1):117-21. [Medline].

  5. Huang MN, Wu CH. Polyarteritis nodosa and antiphospholipid syndrome causing bilateral renal infarction. J Rheumatol. Jan 2009;36(1):197. [Medline].

  6. Bagci P, Erdamar S, Erzin Y, Karatas A, Tuncer M. A case of polyarteritis nodosa diagnosed after recurrent, multiple intestinal perforations. Turk J Gastroenterol. Mar 2009;20(1):71-2. [Medline].

  7. Ventura F, Antunes H, Brito C, Pardal F, Pereira T, Vieira AP. Cutaneous polyarteritis nodosa in a child following hepatitis B vaccination. Eur J Dermatol. May 25 2009;[Medline].

  8. Ebert EC, Hagspiel KD, Nagar M, Schlesinger N. Gastrointestinal involvement in polyarteritis nodosa. Clin Gastroenterol Hepatol. Sep 2008;6(9):960-6. [Medline].

  9. Chan M, Luqmani R. Pharmacotherapy of vasculitis. Expert Opin Pharmacother. Jun 2009;10(8):1273-89. [Medline].

  10. Koc O, Ozbek O, Gumus S, Demir A. Endovascular management of massive gastrointestinal bleeding associated with polyarteritis nodosa. J Vasc Interv Radiol. Feb 2009;20(2):277-9. [Medline].

 
Previous
Next
 
Differential diagnosis of polyarteritis nodosa (PAN) and microscopic polyangiitis (MPA).
Histopathology of kidney of individual with polyarteritis nodosa demonstrates transmural inflammation of arterial wall with heavy infiltrate of polymorphonuclear cells, eosinophils, and mononuclear cells, along with fibrinoid necrosis of inner half of vessel wall.
Histopathology of lung of individual with polyarteritis nodosa demonstrates arterial wall with heavy infiltrate of polymorphonuclear cells, eosinophils, and mononuclear cells and fibrinoid necrosis of vessel wall.
Histopathology of kidney of individual with polyarteritis nodosa demonstrates transmural inflammation of arterial wall with heavy infiltrate of polymorphonuclear cells, eosinophils, and mononuclear cells; fibrinoid necrosis of inner half of vessel wall; and thrombus.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.