Інформація призначена тільки для фахівців сфери охорони здоров'я, осіб,
які мають вищу або середню спеціальну медичну освіту.

Підтвердіть, що Ви є фахівцем у сфері охорони здоров'я.



Журнал "Здоров`я дитини" 8 (59) 2014

Повернутися до номеру

Case report of atypical course of mpo-anca associated vasculitis

Автори: Boyarchuk O.R., Smiyan S.I. - I.Horbachevsky Ternopil State Medical University, Ukraine

Рубрики: Педіатрія/Неонатологія

Розділи: Довідник фахівця

Версія для друку


Classification of vasculitis is one of the most difficult problems in rheumarology [1]. Chapel Hill Сonsensus in 1994 year introduced the concept of ANCA-associated vasculitis because of the involvement of anti-neutrophil cytoplasmic antibodies (ANCA) as the common pathogenesis [2, 3]. Vasculitis associated with antineutrophil cytoplasmic antibodies specific to proteinase 3 (PR3-ANCA)) includes Wegener's granulomatosis, specific to myeloperoxidase (MPO-ANCA) includes microscopic polyangiitis (MPA), idiopathic necrotizing crescentic glomerulonephritis  and Churg-Strauss syndrome [4].  The classic form of MPA is necrotizing vasculitis affecting small vessels. MPA generally presents as a life- and organ-threatening disease. Necrotizing glomerulonephritis is common. Pulmonary capillaritis often occurs [1].  Indolent Wegener’s granulomatosis has been described previously [5].  Two cases of unusually indolent MPO-ANCA has been described in adult [6].

The aim of our study was to show the unusual course of MPO-ANCA associated vasculitis without  renal and lungs involvement.

Case report. We observed a case of indolent course of MPO-ANCA associated vasculitis, with limited progression over few years in girl of 15 years. The current illness began about 6 years ago (in 2008) with recurrent episodes of sinusitis and conjunctivitis, in 2010 – one episode of arthritis and skin hand edema.

In 2012 the episodes were increasing in frequency after streptococcal infection with sinusitis and conjunctivitis, migratory arthritis/arthralgia, skin abnormalities (urticarial rash, angioedema, subcutaneous nodules on elbows, purpura), hoarseness of voice, myalgia, cardiac involvement (extrasystoles), weight loss (5 kg during 1 month). Autoimmune tyroiditis was found. The girl had no pulmonary or renal manifestations. Laboratory examination revealed elevated C-reactive protein level, ESR, ASL-O and seromucoid. The pANCA pattern was detected in titer 1/640, MPO-ANCA by ELISA. ANA was negative, cryoglobulins were not detected, Hep B and C serology was negative, and complement levels—C3 was normal and C4 not much elevated, Ig E, Ig M and Ig G were normal, Ig A - elevated. Urinary sediment and creatinine levels have stayed normal. Skin biopsy revealed infiltration and edema of the dermis, mainly around capillaries, hemorrhage, collagen hyperproduction. The treatment with prednisone 1 mg/kg/day was prescribed.

During the treatment the state of girl has improved. She became more active, decreased clinical signs, MPO-ANCA titre became normal.


Usually MPO-ANCA associated vasculitis occurs in middle-aged adults, but some cases are reported in the pediatric population [7]. The difficulties of diagnosis in this case are in missing data for renal and lungs involvement. This case confirms the possibility of unusually indolent course MPO-ANCA associated vasculitis in children, without involvement of vital organs, that determine the severity of the disease and its consequences.

Due to the rarity of the disease, many countries held a register of all cases of ANCA-associated vasculitis in children. Thus, according to the North American ARChiVe, which included patients from 37 US/ Canadian sites from 2004 to 2010 were registered 155 children with ANCA-associated vasculitis [8], 18 (12%) of them had MPA. The median age at diagnosis was 13 (range 3–16) y. The most common features were renal (95%), constitutional (89%), pulmonary (28%) and sinus-upper respiratory (17%). Anti-MPO were present in 61% of patients.

Early treatment enables a favorable prognosis of MPO-ANCA-associated MPA in children [9].


Despite the fact that the MPO-ANCA associated vasculitis occurring predominantly in middle-aged adults, in rare cases, it is common in children. Multifaceted and low specificity of symptoms makes difficult to diagnose it. We have described a case of the atypical the unusual course of MPO-ANCA associated vasculitis without renal and lungs involvement, but we can not exclude further involvement of the kidneys or lungs, that requiring careful monitoring of these patients.

Список літератури

1.    Cassidy JT, Petty RE. Textbook of Pediatric Rheumatology. Elsevier Saunders, 2005: 614–629.

2.    Van der Woude FJ, Rasmussen N, Lobatto S, Wiik A, Permin H, van Es LA, van der Giessen M, van der Hem GK, The TH. Autoantibodies against neutrophils and monocytes: tool for diagnosis and marker of disease activity in Wegener's granulomatosis. Lancet. 1985 Feb 23;1(8426):425–429. doi: 10.1016/S0140-6736(85)91146-8.

3.    Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL, Hagen EC, Hoffman GS, Hunder GG, Kallenberg CG, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum. 1994 Feb;37(2):187–192. doi: 10.1002/art.1780370206.

4.    Beketova TV, Nasonov EL. Sovremennyie predstavleniya o klassifikatsii i lechenii sistemnyih vaskulitov, assotsiirovannyih s antineytrofilnyimi tsitoplazmaticheskimi antitelami: itogi 2011 g. Terapevticheskiy arhiv. 2012;5:68-74.

5.    Macfarlane DG, Bourne JT, Dieppe PA, Easty DLO. Indolent Wegener’s granulomatosis. Ann Rheum Dis. 1983;4: 398–407.

6.    Gatenby PA. Unusually indolent MPO-ANCA: associated vasculitis—report of two cases. CEN Case Rep. 2013; 2:131–133. doi 10.1007/s13730-012-0045-y.

7.     Briner Verena. Xvoroba Vegenera – systemne zahvoryuvannya. Vnutrennyaya medycyna. 2009;1-2.

8.    Cabral, David A., Uribe, America G., Abramson, Leslie S., Adams, Matthew D., Benseler, Susanne M., Campillo, Sarah S. C., et al; Characterization at Diagnosis of Children with Microscopic Polyangiitis (MPA) Defined Uniquely among Patients with ANCA-Associated Vasculitis (AAV) in a Registry for Children with Vasculitis (ARChiVe). [abstract]. Arthritis Rheum 2010;62 Suppl 10 :1673doi: 10.1002/art.29438.

9.    Peco-Antic A., Bonaci-Nikolic B., Basta-Jovanovic G., Kostic M., Markovic-Lipkovski J., Nikolic M., Spasojevic B. Childhood microscopic polyangiitis associated with MPO-ANCA. Pediatr Nephrol. 2006;21(1):46-53. doi:10.1007/s00467-005-2063-x.

Повернутися до номеру