Pediatric Polyarteritis Nodosa Clinical Presentation

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

History

Classification

Nonspecific signs and symptoms are the hallmarks of polyarteritis nodosa (PAN). In 1990, the American College of Rheumatology (ACR) developed criteria for the classification of PAN. The primary purpose of these guidelines was to define a more homogeneous patient population for research studies and treatment protocols.

The ACR classification of PAN (traditional format) includes the following 10 criteria:

  • Weight loss exceeding 4 kg since illness began, not due to dieting or other factors
  • Livedo reticularis (ie, a mottled reticular pattern) on the skin of portions of the extremities or torso
  • Testicular pain or tenderness that is not due to infection, trauma, or other causes[2]
  • Diffuse myalgias (excluding the shoulder and hip girdle) or weakness of muscles or tenderness of leg muscles[3]
  • Development of mononeuropathy, multiple mononeuropathies, or polyneuropathy
  • Development of hypertension (diastolic blood pressure > 90 mm Hg)
  • Elevation of blood urea nitrogen (BUN) levels above 40 mg/dL or of creatinine levels above 1.5 mg/dL, not due to dehydration or obstruction
  • Presence of hepatitis B surface antigen (HBsAg) or antibody (HBsAb) in serum
  • Arteriogram demonstrating aneurysms or occlusions of the visceral arteries that are not due to arteriosclerosis, fibromuscular dysplasia, or other noninflammatory causes
  • Biopsy of a small or medium-sized artery containing polymorphonuclear (PMN) cells; histologic changes demonstrating the presence of granulocytes or granulocytes and mononuclear leukocytes in the artery wall

For classification purposes, PAN is diagnosed if at least 3 of these 10 criteria are present. The presence of any 3 or more the criteria has a sensitivity of 82.2% and a specificity of 86.6% for PAN.

Symptoms

PAN should be considered whenever certain general and organ-specific symptoms present independently or in combination.

General symptoms associated with PAN include the following:

  • Fever
  • Malaise
  • Weight loss
  • Anorexia

Organ-specific symptoms associated with PAN include the following:

  • Nervous system - Peripheral (mononeuritis multiplex, cranial nerve palsy) and central (motor deficits, strokes, brain hemorrhages) symptoms
  • Skin - Vascular purpura, subcutaneous nodules, livedo reticularis, distal gangrene, and ischemic atrophy[4]
  • Musculoskeletal system - About 50% of patients with PAN have myalgia; the arthritis is an asymmetric, episodic, nondeforming polyarthritis that mainly affects the larger joints, especially in the lower extremities, early in the course of disease in 20% of patients; later in the course of illness, a larger percentage have a more involved polyarticular distribution
  • Kidneys - Rapid renal failure as a consequence of multiple infarcts,[5] renin-dependent hypertension, nephritic syndrome, nephrotic syndrome, rapidly progressive glomerulonephritis (RPGN), and ureteral stenosis may be observed; PAN seldom leads to renal failure, and RPGN is more characteristic of microscopic polyangiitis (MPA)
  • Gastrointestinal (GI) tract - Abdominal pain,[6] digestive tract bleeding, bowel perforation, and malabsorption
  • Cardiopulmonary system - Cardiomegaly, pericarditis, coronary artery involvement leading to ischemia and infarction, pneumonopathy and infiltrates, and pleural effusions
  • Reproductive system - Orchitis in males
  • Eyes - Amblyopia and eye pain
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Physical Examination

General findings include the following:

  • Fever
  • Weight loss

Organ-specific findings may involve the peripheral and central nervous system, the skin,[7] the musculoskeletal system, the kidneys, the GI tract,[8] the cardiopulmonary system, the eyes, and the reproductive system.

Nervous system involvement is seen in as many as 26% of individuals with PAN (though this number may reflect referral patterns unique to the Hospital for Sick Children in the United Kingdom). Peripheral disease (mononeuritis multiplex, cranial nerve palsy, or distal sensorimotor polyneuropathy) has been noted early in 50-70% of patients as a direct result of occlusion of the vasa vasorum. Central disease occurs late and includes motor deficits, strokes, brain hemorrhages, and diffuse encephalopathy.

Skin manifestations occur in approximately 40% of individuals with PAN. Vascular purpura is typically papulopetechial but sometimes may be bullous or vesicular. Infiltration (induration) is not common; however, if it is present, it is the ideal site for biopsy. Subcutaneous nodules, present in 15% of patients with PAN, are transient and should undergo biopsy as soon as they are observed. Livedo reticularis is common. Distal gangrene may be observed as the consequence of ischemia.

One half of patients complain of arthralgia or myalgia. In blind muscle biopsies, 30-50% reveal arteritis.

The kidneys are the organs most frequently affected (63% of individuals with PAN or MPA). In persons with classic PAN, vascular nephropathy is a common manifestation. Renal failure can occur because of multiple infarcts. RPGN is more characteristic of MPA.

Hypertension develops from renal artery involvement. Ureteral involvement, which can be unilateral or bilateral, occurs because of periureteral vasculitis and secondary fibrosis. Intrarenal, perirenal, or retroperitoneal hemorrhage or hematoma results from the rupture of microaneurysms (less frequent in persons with MPA).

GI involvement is reported in 15% of individuals with PAN; however, involvement at autopsy is found in 50%. Abdominal pain can be the first symptom of GI vasculitis. Ischemia most often is in the small intestine; rarely is it found in the colon or stomach. Digestive tract bleeding and bowel perforation can occur.

PAN may present as malabsorption, pancreatitis, or vasculitis of the appendix or gall bladder. Liver involvement, such as infarction and hematoma, can occur in the absence of hepatitis B virus (HBV) infection. Poor prognostic indicators include relapse after surgery or medical treatment, malabsorption, and pancreatitis.

Cardiac involvement occurs in 26% of individuals with PAN and pulmonary involvement in 21%. Cardiomegaly, systolic dysfunction, tricuspid and mitral valve regurgitation, pericarditis, coronary artery involvement leading to ischemia and infarction, pneumonopathy and infiltrates, and pleural effusions are signs of PAN.

Congestive heart failure (CHF) may be present as an indication of coronary arteritis and hypertension. As many as 62% of individuals with PAN have been reported to have coronary artery involvement leading to ischemia and infarction. Cardiac abnormalities are present in 90% of such persons at autopsy.

Retinal vasculitis, retinal detachment, and cotton wool spots (cytoid bodies) are signs of polyarteritis nodosa.

Testicular biopsy reveals vasculitis in 25% of males with PAN.

Clinical characteristics particularly associated with HBV-related PAN include malignant hypertension, renal infarction, and orchiepididymitis.

Limited forms of PAN

Limited forms of PAN include cutaneous PAN, the manifestations of which are well known. Lower extremities are involved most frequently, with palpable purpura, painful nodes surrounded by livedo reticularis, and necrotic lesions. Histologic lesions are identical to those observed in persons with systemic PAN. The outcome is favorable, relapses are frequent, and systemic PAN may develop.

Organs such as the appendix, the gallbladder, the uterus, the testes, and the peripheral nervous system can be involved without systemic involvement.

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Complications

Nervous system complications of PAN include the following:

  • Multiple mononeuropathies
  • Strokes
  • Brain hemorrhages

Cutaneous complications include the following:

  • Ischemia
  • Gangrene
  • Ulcers

Renal complications include the following:

  • Renal failure
  • Hypertension
  • Renal or perirenal hematomas
  • Ureteral stenosis

GI complications include the following:

  • Digestive tract bleeding
  • Bowel perforation
  • Malabsorption
  • Pancreatitis
  • Appendicitis
  • Cholecystitis
  • Liver infarction and hematomas

Cardiopulmonary complications include the following:

  • CHF
  • Atrioventricular block
  • Hypertension
  • Pleural effusion and pneumonopathy

Miscellaneous complications of PAN include the following:

  • Retinal detachment
  • Orchitis
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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].

 
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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.
 
 
 
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