Advertisement

Pediatric Nephrology

, Volume 34, Issue 2, pp 245–247 | Cite as

A child with arthritis, skin rash, abdominal pain and nephritis: searching beyond Henoch–Schönlein purpura—Answers

  • William Chotas
  • Mohammad IlyasEmail author
  • Asad Tolaymat
Clinical Quiz
  • 227 Downloads

Answers

  1. 1.

    Based on clinical presentation, the most likely diagnosis is Henoch–Schönlein purpura (HSP); however, other causes of pauci-immune vasculitis should be considered in the differential diagnosis.

     
  2. 2.

    The renal biopsy showed pauci-immune necrotizing and crescentic glomerulonephritis with global glomerulosclerosis, interstitial fibrosis, and tubular atrophy. The glomerulonephritis demonstrated mild activity and moderate chronicity. There was no evidence of immune complex-mediated disease.

     
  3. 3.

    ANCA testing was performed and she tested positive for IgG C-ANCA (anti-proteinase 3), and negative for myeloperoxidase antibodies.

     
  4. 4.

    Based on renal biopsy and being ANCA positive, the patient was given pulse methylprednisolone, mycophenolate mofetil, and rituximab.

     

Discussion

Henoch–Schönlein purpura is a systemic small-vessel vasculitis affecting 6 to 24 per 100,000 children, who are less than 17 years of age. The disease is characterized by non-thrombocytopenic, palpable purpura...

Keywords

Hematuria Proteinuria Henoch–Schönlein purpura (HSP) Vasculitis ANCA + 

Notes

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

References

  1. 1.
    Pohl M (2015) Henoch–Schönlein purpura nephritis. Pediatr Nephrol 30:245–252CrossRefGoogle Scholar
  2. 2.
    Ozen S, Pistorio A, Iusan SM, Bakkaloglu A, Herlin T, Brik R, Buoncompagni A, Lazar C, Bilge I, Uziel Y, Rigante D, Cantarini L, Hilario MO, Silva CA, Alegria M, Norambuena X, Belot A, Berkun Y, Estrella AI, Olivieri AN, Alpigiani MG, Rumba I, Sztajnbok F, Tambic-Bukovac L, Breda L, Al-Mayouf S, Mihaylova D, Chasnyk V, Sengler C, Klein-Gitelman M, Djeddi D, Nuno L, Pruunsild C, Brunner J, Kondi A, Pagava K, Pederzoli S, Martini A, Ruperto N, Paediatric Rheumatology International Trials Organisation (PRINTO) (2010) EULAR/PRINTO/PRES criteria for Henoch-Schonlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria. Ann Rheum Dis 69:798–806CrossRefGoogle Scholar
  3. 3.
    Lin JJ, Stewart CL, Kaskel FJ, Fine RN (1993) IgG and IgA classes of anti-neutrophil cytoplasmic autoantibodies in a 13-year-old girl with recurrent Henoch-Schonlein purpura. Pediatr Nephrol 7:143–146CrossRefGoogle Scholar
  4. 4.
    Zhang Y, Wu YK, Ciorba MA, Ouyang Q (2008) Significance of antineutrophil cytoplasmic antibody in adult patients with Henoch-Schönlein purpura presenting mainly with gastrointestinal symptoms. World J Gastroenterol 14:622–626CrossRefGoogle Scholar
  5. 5.
    Ozaltin F, Bakkaloglu A, Ozen S, Topaloglu R, Kavak U, Kalyoncu M, Besbas N (2004) The significance of IgA class of antineutrophil cytoplasmic antibodies (ANCA) in childhood Henoch-Schonlein purpura. Clin Rheumatol 23:426–429CrossRefGoogle Scholar
  6. 6.
    Boulis E, Majithia V, Mcmurray R (2013) Adult-onset Henoch–Schonlein purpura with positive c-ANCA (anti-proteinase 3): case report and review of literature. Rheumatol Int 33:493–496CrossRefGoogle Scholar
  7. 7.
    Calatroni M, Oliva E, Gianfreda D, Gregorini G, Allinovi M, Ramirez GA, Bozzolo EP, Monti S, Bracaglia C, Marucci G, Bodria M, Sinico RA, Pieruzzi F, Moroni G, Pastore S, Emmi G, Esposito P, Catanoso M, Barbano G, Bonanni A, Vaglio A (2017) ANCA-associated vasculitis in childhood: recent advances. Ital J Pediatr 43:46CrossRefGoogle Scholar
  8. 8.
    Bui T, Chandrakasan S, Poulik J, Fathalla BM (2013) Granulomatosis with Polyangiitis presenting as Henoch-Schönlein Purpura in children. J Clin Rheumatol 19:199–202CrossRefGoogle Scholar
  9. 9.
    Miyata M, Kanno K, Nishimaki T, Sakuma F, Iwatsuki K, Kasukawa R (2001) A patient with Wegeners granulomatosis with initial clinical presentations of Henoch-Schoenlein Purpura. Intern Med 40:1050–1054CrossRefGoogle Scholar
  10. 10.
    Plumb LA, Oni L, Marks SD, Tullus K (2017) Paediatric anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis: an update on renal management. Pediatr NephrolGoogle Scholar
  11. 11.
    Uribe AG, Huber AM, Kim S, O'Neil KM, Wahezi DM, Abramson L, Baszis K, Benseler SM, Bowyer SL, Campillo S, Chira P, Hersh AO, Higgins GC, Eberhard A, Ede K, Imundo LF, Jung L, Kingsbury DJ, Klein-Gitelman M, Lawson EF, Li SC, Lovell DJ, Mason T, McCurdy D, Muscal E, Nassi L, Rabinovich E, Reiff A, Rosenkranz M, Schikler KN, Singer NG, Spalding S, Stevens AM, Cabral DA, ARegistry for Children with Vasculitis e-entry (ARChiVe) Network (2012) Increased sensitivity of the European Medicines Agency algorithm for classification of childhood granulomatosis with polyangiitis. J Rheumatol 39:1687–1697CrossRefGoogle Scholar
  12. 12.
    Watts R, Lane S, Hanslik T, Hauser T, Hellmich B, Koldingsnes W, Mahr A, Segelmark M, Cohen-Tervaert JW, Scott D (2007) Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies. Ann Rheum Dis 66:222–227CrossRefGoogle Scholar
  13. 13.
    Cabral DA, Canter DL, Muscal E, Nanda K, Wahezi DM, Spalding SJ, Twilt M, Benseler SM, Campillo S, Charuvanij S, Dancey P, Eberhard BA, Elder ME, Hersh A, Higgins GC, Huber AM, Khubchandani R, Kim S, Klein-Gitelman M, Kostik MM, Lawson EF, Lee T, Lubieniecka JM, McCurdy D, Moorthy LN, Morishita KA, Nielsen SM, O'Neil KM, Reiff A, Ristic G, Robinson AB, Sarmiento A, Shenoi S, Toth MB, Van Mater HA, Wagner-Weiner L, Weiss JE, White AJ, Yeung RS, ARChiVe Investigators Network within the PedVas Initiative (2016) Comparing Presenting Clinical Features in 48 Children With Microscopic Polyangiitis to 183 Children Who Have Granulomatosis With Polyangiitis (Wegener's): An ARChiVe Cohort Study. Arthritis Rheumatol 68:2514–2526CrossRefGoogle Scholar
  14. 14.
    Dziuban EJ, Castle VP, Haftel HM (2011) Microscopic polyangiitis in an adolescent presenting as severe anemia and syncope. Rheumatol Int 31:1507–1510CrossRefGoogle Scholar
  15. 15.
    Jindal G, Cruz SD, Punia RP, Kaur R (2011) Refractory Anemia as a presenting feature of microscopic Polyangitis: a rare Vasculitis in children. Indian J Pediatr 78:1287–1289CrossRefGoogle Scholar

Copyright information

© IPNA 2018

Authors and Affiliations

  1. 1.Pediatric Multi-disciplinary ClinicUniversity of FloridaJacksonvilleUSA

Personalised recommendations