title: A novel case of renal pathergy reaction in a Behçet's disease patient complicated by IgA vasculitis.
abstract: A pathergy reaction is defined as a hyperreactivity of the skin in response to minimal trauma, which is important in the diagnosis of Behçet's disease (BD). However, a pathergy reaction may not be restricted to the skin, and little is known about whether an invasive medical procedure can induce the reaction. Here we present a pathergy reaction induced by renal biopsy, an invasive procedure.
  
  
  
  A 46-year-old man who was diagnosed with IgA vasculitis (IgAV) at the age of 38 was treated with prednisolone and mizoribine. However, complications such as common carotid arteritis or recurrent oral ulcer suggested the possibility of another pathophysiology. Later, increasing urine protein developed, suggesting disease aggravation. However, renal biopsy showed arteriosclerotic changes caused mainly by hypertension, negating exacerbation. After renal biopsy, his renal dysfunction and body temperature fluctuated, and detailed examinations revealed recurrent oral and genital ulcers and a folliculitis-like rash on his scrotum. Later, he complained of myodesopsia caused by hemorrhage in the ocular fundus due to occlusive vasculitis. Complete BD was diagnosed after development of the symptoms, and he was treated with prednisolone and colchicine.
  
  
  
  Co-occurrence of BD with IgAV is very rare and may be associated with immune disorders. Interestingly, a renal biopsy revealed BD, which was masked by the presence of IgAV, and elucidated the etiology of the unexplainable symptoms. To the best of our knowledge, this is the first report of renal pathergy. This case enlightens clinicians to the fact that not only a needle stimulation but also an invasive procedure can cause a pathergy reaction.
authors.affiliation: Department of Nephrology, Tokyo Teishin Hospital, 2-14-23, Fujimi, Chiyoda-ku, 102-8798, Tokyo, Japan.
authors.forename: Takaaki
authors.initials: T
authors.lastname: Higashihara
authors.affiliation: Department of Nephrology, Tokyo Teishin Hospital, 2-14-23, Fujimi, Chiyoda-ku, 102-8798, Tokyo, Japan. aokada-tky@umin.ac.jp.|Divison of Nephrology and Endocrinology, The University of Tokyo Graduate School of Medicine, 7-3-1, Hongo, Bunkyo-ku, 113-8655, Tokyo, Japan. aokada-tky@umin.ac.jp.
authors.forename: Akira
authors.identifier: 0000-0001-6480-3388
authors.initials: A
authors.lastname: Okada
authors.affiliation: Department of Nephrology, Tokyo Teishin Hospital, 2-14-23, Fujimi, Chiyoda-ku, 102-8798, Tokyo, Japan.
authors.forename: Taiko
authors.initials: T
authors.lastname: Kusano
authors.affiliation: Department of Allergy and Rheumatology, The University of Tokyo Graduate School of Medicine, 7-3-1, Hongo, Bunkyo-ku, 113-8655, Tokyo, Japan.
authors.forename: Kazuyoshi
authors.initials: K
authors.lastname: Ishigaki
authors.affiliation: Department of Analytic Human Pathology, Graduate School of Medicine, Nippon Medical School, 1-1-5, Sendagi, Bunkyo-ku, 113-8603, Tokyo, Japan.
authors.forename: Akira
authors.initials: A
authors.lastname: Shimizu
authors.affiliation: Department of Nephrology, Tokyo Teishin Hospital, 2-14-23, Fujimi, Chiyoda-ku, 102-8798, Tokyo, Japan.
authors.forename: Hideki
authors.initials: H
authors.lastname: Takano
mesh_terms: D001528:Behcet Syndrome / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology; D003078:Colchicine / Q000627:therapeutic use; D005922:Glomerulonephritis, IGA / Q000150:complications* / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007070:Immunoglobulin A / Q000276:immunology; D007668:Kidney / Q000276:immunology / Q000473:pathology / Q000648:ultrastructure; D008297:Male; D008875:Middle Aged; D011239:Prednisolone / Q000627:therapeutic use; D050257:Tubulin Modulators / Q000627:therapeutic use; D014657:Vasculitis / Q000150:complications* / Q000188:drug therapy / Q000276:immunology
chemical_list: D005938:Glucocorticoids; D007070:Immunoglobulin A; D050257:Tubulin Modulators; D011239:Prednisolone; D003078:Colchicine
keywords: Behçet’s disease; HLA-B51; Henoch-Schönlein purpura; IgA nephritis; IgA vasculitis; Pathergy reaction; Renal biopsy