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Polyarteritis Nodosa, Pediatric

Basics

Description

- Check skin for the following: - Livedo reticularis - Splinter hemorrhages - Erythema nodosum - Necrotic digits

- Urine analysis - Proteinuria and hematuria can be present.

- Infection - Bacterial endocarditis - Brucellosis - Influenza B (calf pain)

- Tumors - Left atrial myxoma - Burkitt lymphoma

- Metabolic - Homocystinuria (thromboembolic events)

- Psychologic - Munchausen syndrome (factitious skin lesions)

- Miscellaneous - Degos disease (malignant atrophic papulosis)

An inflammatory process of small- and medium-sized muscular arteries resulting in dysfunction of affected organs

Epidemiology

Incidence

Extremely rare in childhood

Prevalence

Prevalence equal in boys and girls

Pathophysiology

Necrotizing arteritis of small- and medium-sized arteries resulting in segmental fibrinoid necrosis

Etiology

  • Idiopathic
  • Postinfectious (streptococcal, hepatitis B)

Diagnosis

History

  • Persistent constitutional symptoms
  • Bilateral calf pain
  • Abdominal pain
  • Weight loss
  • Unexplained fever
  • Headache
  • Arthralgia/myalgia
  • Rashes
  • Seizures
  • Weakness

Physical Exam

  • Check skin for the following:Livedo reticularisSplinter hemorrhagesErythema nodosumNecrotic digits
  • Assess BP and pulses.
  • Neurologic exam for findings consistent with neuropathy (mononeuritis multiplex)
  • Ophthalmologic exam for cotton wool spots
  • Check testes for tenderness or swelling.
  • Check muscles for tenderness, especially calves.

Diagnostic Tests & Interpretation

Lab

Initial Lab Tests

  • ESRUsually extremely elevated; leukocytosis and thrombocytosis are seen.
  • Urine analysisProteinuria and hematuria can be present.
  • Creatinine and BUN levels
  • Antinuclear antibodies and rheumatoid factor
  • Muscle enzymes (creatine kinase, lactate dehydrogenase, aspartate aminotransferase, and aldolase levels)Muscle involvement is common, especially in those with calf pain.
  • Antineutrophil cytoplasmic antibodies (ANCAs)Detectable in some; usually perinuclear (p), rarely cytoplasmic (c) staining patterns; ANCA is more commonly associated with other vasculitides:pANCA pattern usually caused by anti-myeloperoxidase (MPO) antibodies; also seen in microscopic polyangiitiscANCA pattern usually caused by anti-proteinase 3 (PR3) antibodies; also seen in granulomatous with polyangiitis (Wegener granulomatosis)
  • Hepatitis B serologiesHepatitis B has been associated in some series of patients with polyarteritis nodosa.
  • Streptococcal titersPolyarteritis nodosa may develop after streptococcal infections.

Alert

The detection of ANCA, previously thought to be highly specific for vasculitis, now appears to be less so. Hence, it remains important to confirm the diagnosis of polyarteritis nodosa with biopsy or angiography.

Imaging

  • MRI of tender musclesShort T1 inversion recovery (STIR) images may show edema, so a directed biopsy can be done to avoid false-negative muscle biopsy.
  • MRA, CT angiography, or angiographyCan demonstrate vessel wall stenoses and aneurysm

Diagnostic Procedures/Other

Biopsy of affected tissue/organ: usually skin, kidney, nerve, testicle

Differential Diagnosis

  • InfectionBacterial endocarditisBrucellosisInfluenza B (calf pain)
  • TumorsLeft atrial myxomaBurkitt lymphoma
  • MetabolicHomocystinuria (thromboembolic events)
  • Congenital
  • ImmunologicSystemic necrotizing vasculitisSystemic lupus erythematosusKawasaki diseaseSystemic juvenile idiopathic arthritisGranulomatosis with polyangiitis (Wegener granulomatosis)Takayasu arteritisCryoglobulinemiaAntiphospholipid antibody syndromeThrombotic thrombocytopenic purpura
  • PsychologicMunchausen syndrome (factitious skin lesions)
  • MiscellaneousDegos disease (malignant atrophic papulosis)

Treatment

Medication

  • Corticosteroids are mainstay.Usually start at dose of 1 " 2 mg/kg/24 h and adjust based on response.May initially give methylprednisolone 30 mg/kg up to 1 g/24 h IV once daily for 3 days
  • Immunosuppressives such as methotrexate, azathioprine, and cyclophosphamide may be necessary.
  • Hypertension should be managed aggressively.

Additional Treatment

General Measures

  • Medication
  • Diet
  • CautionDo not initiate therapy before efforts to establish the diagnosis.

Ongoing Care

Follow-up Recommendations

  • Initiation of steroid therapy may bring response in 1 " 2 weeks; however, management of specific organs affected during acute stage is essential.
  • May require long-term therapy

Patient Monitoring

  • Watch for the following:Rising creatinine and BUN levelsAbdominal painUncontrolled hypertension
  • Home testingMay wish to have patients monitor BP periodically if renal involvement suspected

Diet

  • If renal system involved, diet low in sodium and potassium
  • Possible conflicts with medications

Prognosis

  • May be extremely poor over the long term
  • Risk is high for renal failure, hypertension, stroke, myocardial infarction, bowel infarction, and death.
  • Owing to low incidence/prevalence, precise data are not available.
  • Cutaneous polyarteritis nodosa is relatively benign.

Complications

  • Hypertension
  • Renal failure
  • Digital necrosis
  • Intestinal infarction
  • Stroke

Additional Reading

  • Eleftheriou D, Dillon MJ, Tullus K, et al. Systemic polyarteritis nodosa in the young: a single centre experience over thirty-two years. Arthritis Rheum. 2013;65(9):2476 " 2485. doi:10.1002/art.38024 [View Abstract]
  • Kawakami T. A review of pediatric vasculitis with a focus on juvenile polyarteritis nodosa. Am J Clin Dermatol. 2012;13(6):389 " 398. [View Abstract]
  • Khubchandani RP, Viswanathan V. Pediatric vasculitides: a generalists approach. Indian J Pediatr. 2010;77(10):1165 " 1171. [View Abstract]
  • Morgan AJ, Schwartz RA. Cutaneous polyarteritis nodosa: a comprehensive review. Int J Dermatol. 2010;49(7):750 " 756. [View Abstract]
  • Ozen S, Anton J, Arisoy N, et al. Juvenile polyarteritis: results of a multicenter survey of 110 children. J Pediatr. 2004;145(4):517 " 522. [View Abstract]
  • Ting TV, Hashkes PJ. Update on childhood vasculitides. Curr Opin Rheumatol. 2004;16(5):560 " 565. [View Abstract]

Codes

ICD09

  • 446.0 Polyarteritis nodosa

ICD10

  • M30.0 Polyarteritis nodosa

SNOMED

  • 155441006 Polyarteritis nodosa (disorder)
  • 239926000 Cutaneous polyarteritis nodosa
  • 239925001 Polyarteritis nodosa with single organ involvement
  • 239927009 Polyarteritis nodosa with multi-organ involvement

FAQ

  • Q: When should I consider polyarteritis nodosa in the differential?
  • A: There are 5 major clues to polyarteritis nodosa: (1) prolonged constitutional symptoms without a diagnosis, (2) multisystem disease, (3) an unusual patient for the presenting symptom (myocardial infarction in a teen), (4) a rash that looks vasculitic, and (5) bilateral calf pain in a sick child.
  • Q: What is the difference between polyarteritis nodosa and systemic necrotizing vasculitis?
  • A: Polyarteritis nodosa has a strict definition. Many children who clearly have vasculitis of the small- and medium-sized arteries do not fit precisely into the description of polyarteritis nodosa. In most ways, the search for organ involvement and therapy is the same.
  • Q: Who should manage the patient with polyarteritis nodosa?
  • A: Usually, one discipline provides comprehensive management plan (either the pediatrician or rheumatologist). Subspecialist(s) of the affected organ systems provide management guidelines for specific organ issues.