Clinical Rheumatology

, Volume 38, Issue 11, pp 3311–3313 | Cite as

Less IgA deposits with more severe disease: the immunoclinical paradox in Henoch-Schönlein Purpura with MEFV mutations

  • Ufuk İlgenEmail author
  • Gökhan Nergizoğlu
Letter to the Editor

Dear Editor,

We have read the article by Cakici et al. [ 1] with interest and would like to discuss the association of histopathological findings with MEditerranean Fe Ver gene ( MEFV) mutations further. The authors reported that 38.9% vs 94% of the Henoch-Schönlein Purpura (HSP) patients with and without MEFV mutations, respectively, had immunoglobulin A (IgA) deposits on skin or renal biopsy. Previous reports of HSP cases without overt IgA deposits in patients with Familial Mediterranean Fever (FMF) supported their findings. Still, MEFV mutations, particularly the pathogenic exon 10 variants, were reported to be associated with a severe HSP phenotype [ 1]. Importantly, not the presence or intensity of immunostaining of IgA, but proliferative changes, glomerulosclerosis, tubular atrophy, and interstitial fibrosis, which are direct consequences of the inflammatory process, are prognostic in both HSP nephritis and a closely related, histopathologically indistinguishable entity, IgA...


Compliance with ethical standards


Expanded data from a previously published work were used here with permission from İlgen U, Nergizoğlu G. MEditerranean FeVer (MEFV) gene mutations in glomerulonephritides: a clinicopathological study. Turkish Journal of Medical Sciences 48(6). Copyright© 2018.

Informed consent

Written informed consent was obtained from patients participated in this study.

Ethical approval

Institutional Review Board of Ankara University Medical School approved this study.




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Copyright information

© International League of Associations for Rheumatology (ILAR) 2019

Authors and Affiliations

  1. 1.Department of RheumatologyTrakya University Medical SchoolEdirneTurkey
  2. 2.Department of NephrologyAnkara UniversityAnkaraTurkey

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