Systematic review of childhood-onset polyarteritis nodosa and DADA2

Cüceoğlu, Müşerref Kasap; Şener, Seher; Batu, Ezgi Deniz; Akça, Ümmüşen Kaya; Sağ, Erdal; Atalay, Erdal; Balık, Zeynep; Başaran, Özge; Bilginer, Yelda; Özen, Seza

doi:10.1016/j.semarthrit.2021.04.009

Cited by 16 publications

(17 citation statements)

References 62 publications

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“…In the MYPAN trial, remission at 6 months of treatment was noted in 4 of 6 patients on MMF as compared with 4 of 5 patients on CYC therapy. In a recently published study from Turkey, 3 patients received MMF as induction therapy (included in MYPAN trial); however, the authors noted these patients to have a lower disease activity as compared with the rest of their c-sPAN cohort 5. The same study group has previously noted lower CRP levels and lower frequency of weight loss in c-sPAN as compared with adult systemic PAN (a-sPAN) 6.…”

Section: Discussionmentioning

confidence: 99%

Mycophenolate mofetil in the treatment of childhood systemic polyarteritis nodosa

Bhattarai¹,

Banday

Nori

et al. 2022

BMJ Case Rep

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Polyarteritis nodosa (PAN) is a medium vessel vasculitis with necrotising vascular changes along with multisystemic involvement. Due to variable initial presentations, diagnosis of systemic PAN in children requires a comprehensive work up. In addition, systemic PAN needs an aggressive therapy. Mycophenolate mofetil is an emerging newer alternative for the treatment of PAN. We report a case of childhood systemic PAN who initially presented with subtle signs like reduced sensation over lateral foot, non-deforming arthritis and multiform rashes. After comprehensive aetiological work up, nerve biopsy and supporting evidence clinched the diagnosis. Vasculitis in children presenting with benign subtle signs is sometimes a diagnostic challenge to clinicians. Our case highlights the importance of lateral thinking while dealing with non-specific multisystemic signs. Evidence of successful treatment of PAN with mycophenolate mofetil is gradually being built up. It is also described to result lower relapse and increased treatment free survival rate.

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Section: Discussionmentioning

confidence: 99%

Mycophenolate mofetil in the treatment of childhood systemic polyarteritis nodosa

Bhattarai¹,

Banday

Nori

et al. 2022

BMJ Case Rep

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“…TCZ was favoured over a TNF-blocker due to high acute phase reactants (indicating increased inflammation) and markedly elevated IL-6 levels (relatively higher compared to TNF-alpha level elevation). Indeed, TNF-inhibitors may be the primary choice in patients in which ADA2 deficiency cannot rapidly be excluded as was the case in our patient by negative genetic testing and normal serum ADA2 activity; TNF-inhibitors have been successfully used in children with PAN and ADA2 deficiency [ 6 , 7 ].…”

Section: Discussionmentioning

confidence: 99%

A young girl with severe polyarteritis nodosa successfully treated with tocilizumab: a case report

et al. 2021

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Background Childhood Polyarteritis nodosa (PAN) is a systemic vasculitis with necrotizing inflammation of medium- and small-sized arteries. Disease evolution may be severe and refractory to standard treatment including prednisone, azathioprine and cyclophosphamide. Case presentation We present the case of a young girl with severe PAN resulting in progressive ischemia and necrosis of fingers and toes. Biological work-up revealed increased acute phase reactants and interleukin-6 levels. She was only partially controlled despite high-dose corticosteroids and cyclophosphamide infusions, and eventually achieved rapid improvement and sustained remission on tocilizumab. Further, we review the current evidence of the interleukin-6-inhibitor tocilizumab for the treatment of PAN. Conclusion Tocilizumab may be an efficient therapeutic option in a subset of treatment-refractory children with PAN.

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“…Общий анализ крови: эритроциты -5,34×10 12 /л (3,7-4,7×10 12 /л), гемоглобин -128 г/л (110-152 г/л), лейкоциты -16,2×10 9 л ((4-10)×10 9 /л), эозинофилы -0,2% (0,5-7%), лимфоциты -39,8% (20-40%), моноциты -5,2% (1-6%), СОЭ -5 мм/ч (1-8 мм/ч).…”

Section: лабораторные исследования (выполнены по прибытии пациента в ...unclassified

“…Общий анализ крови: эритроциты 3,83×10 12 /л ((3,5-4,7)×10 12 /л), гемоглобин 107 г/л (110-145 г/л), лимфоциты 17% (24-54%).…”

Section: лабораторные исследования (выполнены при поступлении в гбуз ...unclassified

“…При тяжелом течении УП применяется комбинация препаратов глюкокортикоидов и циклофосфамидов, а также сочетание с сеансами плазмафереза [12]. Но важно отметить, что назначение комбинированных препаратов способно приводить к развитию нежелательных побочных эффектов, которые развиваются в первый год у 25% пациентов, а лечение пациентов иммуносупрессивными препаратами может оказывать токсическое действие и повышать риск инфекционных и онкологических заболеваний [13].…”

Section: Introductionunclassified

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Polyarteritis Nodosa: Clinical Cases in Boys 12 and 9 Years Old

Burlutskaya

Савельева

Ustyuzhaninа

et al. 2023

Kuban. nauсh. med. vestnik

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Background. Polyarteritis nodosa is an acute, subacute or chronic immune complex disease associated with peripheral and visceral artery involvement, predominantly of middle and small sizes, development of destructive-proliferative arteritis and subsequent peripheral and visceral ischaemia.Cases description. The present paper describes two clinical cases of polyarteritis nodosa in patient R., aged 12, and patient A., aged 9, and demonstrates the difficulties of diagnosing the disease in its early stages. Patient R., aged 12, was admitted to the Rheumatology Unit of the Krasnodar Krai Children’s Clinical Hospital with complaints of red, patchy, dense rash on the palms and plantar surface of the feet. The child has been ill since September 2017, and after a history of tonsillitis suffered a fever of 37 °C, pain in the right heel area, nodular thickening on the feet, livedo reticularis, swelling of both hands. The disease had a recurrent course. The boy was treated with prednisolone, mycophenolate mofetil, hydroxychloroquine and three courses of rituximab (April 2018, January 2019, September 2020). Repeated courses of human normal immunoglobulin and alprostadil therapy were carried out. The treatment showed positive dynamics, fever was eliminated, general well-being improved, and acute inflammatory markers in blood became normal. The skin retained minimal manifestation of livedo, nodularities on the feet did not progress in dynamics. Patient A. was admitted to the Rheumatology Unit of the Krasnodar Krai Children’s Clinical Hospital in April 2022 with complaints of weakness, myalgia of the lower extremities and necrosis foci in the left lumbar region. The medical history indicates that in March 2022, the boy, being in good health before, developed a bluish, painful rash on his lower legs after a workout. Skin changes and soreness resolved on their own without treatment. After examination, a diagnosis was made as follows: juvenile polyarteritis nodosa, activity score — 3.Conclusion. The diagnosis of polyarteritis nodosa can be often problematic due to the very character of the disease featured by absence of specific symptoms, by polymorphism of clinical manifestations, and by lack of clear diagnostic and laboratory markers.

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Systematic review of childhood-onset polyarteritis nodosa and DADA2

Cited by 16 publications

References 62 publications

Mycophenolate mofetil in the treatment of childhood systemic polyarteritis nodosa

Mycophenolate mofetil in the treatment of childhood systemic polyarteritis nodosa

A young girl with severe polyarteritis nodosa successfully treated with tocilizumab: a case report

Polyarteritis Nodosa: Clinical Cases in Boys 12 and 9 Years Old

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