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Childhood Polyarteritis Nodosa: A Prospective Multicentre Study from Eastern India

The Indian Journal of Pediatrics volume 81, pages 371–374 (2014)Cite this article

Abstract

Objective

To delineate the spectrum of clinical presentation and system involvement in childhood Polyarteritis Nodosa (PAN) in a multicentre follow up.

Methods

This prospective observational study included all children less than 12 y attending pediatric rheumatological clinic of four different institutes in West Bengal or admitted with rheumatological symptoms and were diagnosed as childhood PAN by ACR and EULAR/PRES/PRINTO criteria. During the study period of 8 y, 15 cases were categorized as childhood PAN with organ involvement. The children diagnosed as microscopic polyangiitis or cutaneous PAN were excluded.

Results

The mean age of presentation was 8.6 ± 2.09 y with male to female ratio of 7:8. All had prolonged fever, severe myalgia, skin involvement and elevated acute phase reactants. Hypertension (93 %) and peripheral neuropathy (46 %) were notable findings. Nine had typical peripheral gangrene with ulceration, whereas rash of livedo reticularis was seen in 6 patients. None had renal, pulmonary or testicular involvement. Diagnosis was established in all patients by skin biopsy which showed necrotizing vasculitis of the medium sized vessels. The clinical presentations and diagnostic parameters were compared between the genders and no statistically significant difference was noted. All patients required corticosteroids while 10 patients needed additional immunosuppressives where there were uncontrolled symptoms of peripheral neuropathy or progressive gangrene despite treatment with corticosteroids. Cyclophosphamide was used most commonly (n = 7). Two required methyl prednisolone. Two children were lost to follow up. One patient had two relapses within three years. No mortality was observed.

Conclusions

This multicentric study on childhood PAN, first of its kind from India, demonstrated a distinctive pattern of system involvement and clinical presentations.

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Contributions

RM: Conception and design of the study. RM, SS, PP, MN, TKS helped in patient management and data collection. RM, SS, AH: Drafting, analysis and interpretation of data. RM, SS: Searched the literature and substantially revised it. All authors finally approved the manuscript.

Conflict of Interest

None.

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None.

Author information

Affiliations

  1. Pediatric Rheumatology Clinic, Department of Pediatric Medicine, North Bengal Medical College Hospital (NBMCH), Darjeeling, India

    Rakesh Mondal

  2. Pediatric Rheumatology Clinic, Department of Pediatric Medicine, IPGME&R, Kolkata, India

    Sumantra Sarkar

  3. Pediatric Rheumatology Clinic, Department of Pediatrics, Institute of Child Health, Kolkata, India

    Priyankar Pal

  4. Pediatric Nephrology Clinic, Department of Pediatric Medicine, IPGME&R, Kolkata, India

    Madhumita Nandi

  5. Department of Pharmacology, IPGME&R, Kolkata, India

    Avijit Hazra

  6. Pediatric Rheumatology Clinic, Department of Pediatrics, NRS Medical College and Hospital, Kolkata, India

    Tapas Kumar Sabui

  7. Balarampur, Mahestala (PO), Kolkata, 700141, India

    Rakesh Mondal

Authors
  1. Rakesh Mondal
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  2. Sumantra Sarkar
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  3. Priyankar Pal
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  4. Madhumita Nandi
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  5. Avijit Hazra
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  6. Tapas Kumar Sabui
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Corresponding author

Correspondence to Rakesh Mondal.

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Mondal, R., Sarkar, S., Pal, P. et al. Childhood Polyarteritis Nodosa: A Prospective Multicentre Study from Eastern India. Indian J Pediatr 81, 371–374 (2014). https://doi.org/10.1007/s12098-013-1256-8

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  • DOI: https://doi.org/10.1007/s12098-013-1256-8

Keywords

  • Polyarteritis nodosa
  • Children
  • Clinical spectrum
  • India