DOI: http://dx.doi.org/10.18203/2349-3291.ijcp20200702

Clinicopathological and morbidity profile of childhood polyarteritis nodosa in a tertiary referral center

Sanjay Kantharajpura Shivappa, Jessica Laltlansangi Hlawndo, Pradeep Rudramurthy, Jyothi Raghuram, Anand Prahlad Rao

Abstract


Background: Polyarteritis nodosa (PAN) is a primary systemic necrotizing vasculitis predominantly targeting medium-sized arteries defined as the main visceral arteries and their branches. The objective of this study was to analyze the epidemiology, clinico-pathological and point outcome of PAN in children at a tertiary care centre in Karnataka.

Methods: This prospective observational study was conducted among eighteen children diagnosed with PAN from December 2015 to May 2017.  All the children fulfilling EULAR/PRES/PRINTO classification criteria for childhood PAN were included in this study. Clinico-pathological profile of these patients was studied and documented.

Results: This study had shown that there are two peaks of age at onset of symptoms at 5 to 7 years and from 11 to 13 years. No sex predilection was noted. Most children present with nonspecific symptoms like myalgia (94%), fever (84%), weight loss (50%) and joint pain (50%). Skin manifestations were seen in all the children. Neurological involvement (61%) in the form of mononeuritis multiplex (38.8%), hypertensive encephalopathy (11%) and stroke (11%) was observed. Four children (22%) presented with pain abdomen while two boys had testicular pain. Only one child had renal manifestation in the form of glomerulonephritis. Most children had neutrophilic leukocytosis, elevated ESR and thrombocytosis. Skin biopsy was performed in ten children and out of these, 9 reports were suggestive of PAN. Angiography was done in four children revealing multiple aneurysms or stenosis in the systemic arteries. All the children received corticosteroids. Cyclophosphamide was used as an induction agent in about 50% of the patients. Corticosteroid treatment alone was sufficient in only one patient. Steroid sparing agent like azathioprine (38%), mycophenolate mofetil (33%) and hydroxychloroquine (5%) were used with low dose steroids as maintenance therapy. Seven children (39%) achieved remission off drugs while 8 children are still on treatment, one child relapsed and three children were lost to follow up.

Conclusions: Childhood vasculitis leads to considerable delay in diagnosis the initiation of treatment. High index of suspicion and early referral is required in all cases of vasculitis to reduce morbidity and mortality.


Keywords


Aneurysms, Inflammation, Multisystem disease, Vasculitis

Full Text:

PDF

References


Khubchandani RP, Viswanathan V. Pediatric vasculitides: a generalists approach. Ind J Pediatr. 2010 Oct 1;77(10):1165-71.

Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, Flores‐Suarez LF, Gross WL, Guillevin L, Hagen EC, Hoffman GS. 2012 revised international chapel hill consensus conference nomenclature of vasculitides. Arthr Rheumat. 2013 Jan;65(1):1-1.

Ozen S, Anton J, Arisoy N, Bakkaloglu A, Besbas N, Brogan P, et al. Juvenile polyarteritis: results of a multicenter survey of 110 children. J Pediatr. 2004 Oct 31;145(4):517-22.

Mogale KD, Shrivastava A. Childhood polyarteritis nodosa: a clinical diagnosis. Ind Pediatr. 2006;43(10): 911-3.

Watts RA, Scott DG. Epidemiology of vasculitis. In: Ball GV, Bridges SL, editors. Vasculitis. 2nd ed. Oxford: Oxford University Press; 2008:7-21.

Dillon MJ, Eleftheriou D, Brogan PA. Medium-size-vessel vasculitis. Pediatr Nephrol. 2010;25:1641-52.

Ettlinger RE, Nelson AM, Burke EC, Lie JT. Polyarteritis nodosa in childhood: a clinical pathologic study. Arthritis Rheum. 1979;22:820-5.

Besbas N, Ozen S, Saatci U, Topalogclu R, Tinaztepe K, Bakkaloglu A. Renal involvement in polyarteritis nodosa: evaluation of 26 Turkish children. Pediatr Nephrol. 2000;14:325-7.

Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL, et al. Nomenclature of systemic vasculitides. Arthr Rheumat: Official J Am Coll Rheumatol. 1994 Feb;37(2):187-92.

Arend WP, Michel BA, Bloch DA, Hunder GG, Calabrese LH, Edworthy SM, et al. The American College of Rheu¬matology 1990 criteria for the classification of Takayasu arteritis. Arthr Rheum 1990;33:1129-34.

Leavitt RY, Fauci AS, Bloch DA, Michel BA, Hunder GG, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Wegener’s granulomatosis. Arthritis Rheum. 1990;33:1101-7.

Lightfoot RW, Jr., Michel BA, Bloch DA, Hunder GG, Zvaifler NJ, McShane DJ, et al. The American College of Rheumatology 1990 criteria for the classification of polyarteritis nodosa. Arthritis Rheum. 1990;33:1088-93.

Mills JA, Michel BA, Bloch DA, Calabrese LH, Hunder GG, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Henoch- Schonlein purpura. Arthritis Rheum. 1990;33:1114-21.

Ozen S, Pistorio A, Iusan SM, Bakkaloglu A, Herlin T, Brik R, et al. EULAR/PRINTO/PRES criteria for Henoch–Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: final classification criteria. Ann Rheum Dis. 2010 May 1;69(5):798-806.

Kumar L, Sarkar B, Singh S, Bajwa RP, Joshi K, Malik N. Polyarteritis nodosa-a few unusual findings. Indian Pediatr. 1996 Jun 1;33:459-64.

Mondal R, Sarkar S, Pal P, Nandi M, Hazra A, Sabui TK. Childhood polyarteritis nodosa: a prospective multicentre study from eastern India. Ind J Ped. 2014 Apr 1;81(4):371-4.

Maeda M, Kobayashi M, Okamoto S, Fuse T, Matsuyama T, Watanabe N, et al. Clinical observation of 14 cases of childhood polyarteritis nodosa in Japan. Pediatr Inter. 1997 Apr 1;39(2):277-9.

Blau EB, Morris RF, Yunis EJ. Polyarteritis nodosa in older children. Pediatr. 1977 Aug 1;60(2):227-34.

Jelusic M, Batinic D, Milosevic D, Malenica B, Malcic I. Polyarteritis nodosa in Croatian children: a retrospective study over the last 20 years. Rheumatol Inter. 2013 Dec 1;33(12):3087-90.

Ettlinger RE, Nelson AM, Burke EC, Lie JT. Polyarteritis nodosa in childhood. Arthr Rheumatol. 1979 Aug 1;22(8):820-5.

Eleftheriou D, Dillon MJ, Tullus K, Marks SD, Pilkington CA, Roebuck DJ, et al. Systemic Polyarteritis Nodosa in the Young: A Single-Center Experience Over Thirty-Two Years. Arthr Rheumat. 2013;65(9):2476-85.

Ozen S, Bakkaloglu A, Dusunsel R, Soylemezoglu O, Ozaltin F, Poyrazoglu H, et al. Childhood vasculitides in Turkey: a nationwide survey. Clini Rheumatol. 2007 Feb 1;26(2):196-200.

Talukder MK, Islam MI, Rahman SA. Clinical and laboratory profile of childhood polyarteritis nodosa in a Bangladeshi tertiary hospital. Inter J Rheumat Dis. 2014 Mar 1;17(3):313-6.

Iudici M, Puéchal X, Pagnoux C, Quartier P, Agard C, Aouba A, et al. Brief Report: Childhood‐Onset Systemic Necrotizing Vasculitides: Long‐Term Data From the French Vasculitis Study Group Registry. Arthr Rheumatol. 2015 Jul 1;67(7):1959-65.