Medical Care

Medical care of the patient with polyarteritis nodosa (PAN) is individualized. In the acute phase, hospitalization usually is required. Diagnostic tests, imaging studies, and biopsies as appropriate are carried out expeditiously. Intensive care often is necessary to provide for frequent monitoring and emergency medical or surgical treatment.

Inpatient and outpatient care in childhood polyarteritis nodosa (CPAN) is individualized for each patient. Medications are tailored to the patient's needs and disease course.

Surgical Care

Patients with polyarteritis nodosa (PAN) may develop acute abdominal emergencies, including intractable GI bleeding, bowel infarction, and viscus or aneurysmal rupture. These complications are associated with significant mortality. The author has treated a preadolescent boy who underwent coronary artery bypass surgery for the management of myocardial ischemia and necrosis secondary to PAN.

Histologic confirmation is necessary to make the diagnosis of PAN. Biopsies of muscle, sural nerve, kidney, liver, testis, and rectum have been used to confirm PAN and are preferred to biopsies of skin, which are less diagnostic and do not necessarily indicate the extent of systemic involvement.

A: Right coronary artery stenosis. B: Stent in place across stenotic segment.

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Close-up of stent in place.

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Consultations

Specialty consultations depend on the individual patient's disease onset and course. A pediatric rheumatologist experienced in the treatment of children with systemic vasculitis should head the team and direct the overall care of infants and children with childhood polyarteritis nodosa (CPAN). Pediatric cardiologists, critical care specialists, neurologists, surgeons, physiatrists, pulmonologists, gastroenterologists, and nephrologists may need to be consulted at times.

Diet

Diet is dictated by the extent and involvement of the disease and by its therapy.

Activity

Activity is based on the patient's tolerance. In general, the child is encouraged to increase physical activity.

Guidelines

Cannon L, Wu EY. Recent Advances in Pediatric Vasculitis. Rheum Dis Clin North Am. 2021 Nov. 47 (4):781-96. [QxMD MEDLINE Link]. [Full Text].
Bloch DA, Michel BA, Hunder GG, et al. The American College of Rheumatology 1990 criteria for the classification of vasculitis. Patients and methods. Arthritis Rheum. 1990 Aug. 33(8):1068-73. [QxMD MEDLINE Link].
Gocke DJ, Hsu K, Morgan C, Bombardieri S, Lockshin M, Christian CL. Association between polyarteritis and Australia antigen. Lancet. 1970 Dec 5. 2(7684):1149-53. [QxMD MEDLINE Link].
Duffy J; Lidsky MD; Sharp JT; Davis JS; Person DA; Hollinger FB; Min KW. Polyarthritis, polyarteritis and hepatitis B. Medicine (Baltimore). 1976 Jan. 55(1):19-37. [QxMD MEDLINE Link].
Mossberg M, Segelmark M, Kahn R, Englund M, Mohammad AJ. Epidemiology of primary systemic vasculitis in children: a population-based study from southern Sweden. Scand J Rheumatol. 2018 Feb 7. 1-8. [QxMD MEDLINE Link].
Guirola R, Hunter JV, Perez M, Muscal E. Childhood polyarteritis nodosa presenting with central nervous system manifestations and the posterior reversible encephalopathy syndrome. J Child Neurol. 2014 Jan. 29 (1):103-7. [QxMD MEDLINE Link].
Beckum KM, Kim DJ, Kelly DR, Weiser P, Lara DA, Theos A. Polyarteritis nodosa in childhood: recognition of early dermatologic signs may prevent morbidity. Pediatr Dermatol. 2014 Jan-Feb. 31 (1):e6-9. [QxMD MEDLINE Link].
[Guideline] Chung SA, Gorelik M, Langford CA, et al. 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Polyarteritis Nodosa. Arthritis Rheumatol. 2021 Aug. 73 (8):1384-93. [QxMD MEDLINE Link]. [Full Text].
Boistault M, Lopez Corbeto M, Quartier P, Berbel Arcobé L, Carsi Durall A, Aeschlimann FA. A young girl with severe polyarteritis nodosa successfully treated with tocilizumab: a case report. Pediatr Rheumatol Online J. 2021 Dec 3. 19 (1):168. [QxMD MEDLINE Link]. [Full Text].
Do N, Ringold S, Brandling-Bennett H. Cutaneous polyarteritis nodosa in pediatric patients successfully treated with TNF-α inhibitor and methotrexate: Case series and literature review. Pediatr Dermatol. 2019 Nov. 36 (6):932-5. [QxMD MEDLINE Link].
Belay ED, Maddox RA, Holman RC, Curns AT, Ballah K, Schonberger LB. Kawasaki syndrome and risk factors for coronary artery abnormalities: United States, 1994-2003. Pediatr Infect Dis J. 2006 Mar. 25(3):245-9. [QxMD MEDLINE Link].
Googe JM Jr, Brady SE, Argyle JC, Apple DJ, Gooch WM 3rd. Choroiditis in infantile periarteritis nodosa. Arch Ophthalmol. 1985 Jan. 103(1):81-3. [QxMD MEDLINE Link].
Hirose S, Hamashima Y. Morphological observations on the vasculitis in the mucocutaneous lymph node syndrome. A skin biopsy study of 27 patients. Eur J Pediatr. 1978 Aug 17. 129(1):17-27. [QxMD MEDLINE Link].
Jennette JC, Falk RJ. Small-vessel vasculitis. N Engl J Med. 1997 Nov 20. 337(21):1512-23. [QxMD MEDLINE Link].
Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum. 1994 Feb. 37(2):187-92. [QxMD MEDLINE Link].
Kallenberg CGM. Taxonomy of the vasculitis syndromes. Van de Putte LBA, Furst DE, Williams HJ, van Riel PLCM, eds. Therapy of Systemic Rheumatic Disorders. New York: Marcel Dekker, Inc; 1998. 563-570.
Lie JT. Biopsy Diagnosis in Rheumatic Diseases. New York: IGAKU-SHOIN; 1997. 61-106: 175-179.
Lie JT. Illustrated histopathologic classification criteria for selected vasculitis syndromes. American College of Rheumatology Subcommittee on Classification of Vasculitis. Arthritis Rheum. 1990 Aug. 33(8):1074-87. [QxMD MEDLINE Link].
Lightfoot RW Jr, Michel BA, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of polyarteritis nodosa. Arthritis Rheum. 1990 Aug. 33(8):1088-93. [QxMD MEDLINE Link].
Long SS. Fifty years ago in the Journal of Pediatrics. J Pediatr. 1999. 135:171.
Matteson EL. A history of early investigation in polyarteritis nodosa. Arthritis Care Res. 1999 Aug. 12(4):294-302. [QxMD MEDLINE Link].
Person DA. Vasculitis. Case Based Pediatrics for Medical Students and Residents. August 2002. Chapter XVI.3:
Person DA, He X, Brewer EJ Jr. Vasculitis in children with juvenile rheumatoid arthritis. Hick RV. Vasculopathies of Childhood. 1988. 156-160.

Media Gallery

Childhood polyarteritis nodosa. Bilateral axillary aneurysms.
Childhood polyarteritis nodosa. Gangrene of the forearm and lower leg in a 3-month-old infant.
Childhood polyarteritis nodosa. Multiple aneurysms on arteriogram and autopsy specimen.
Thrombosed cystic artery in childhood polyarteritis nodosa.
Childhood polyarteritis nodosa. Heart with 5 coronary aneurysms.
A: Right coronary artery stenosis. B: Stent in place across stenotic segment.
Close-up of stent in place.