Multicentric Castleman's disease associated with IgA vasculitis (Henoch-Schönlein purpura) responding well to tocilizumab: a case report.
A 41-year-old man was referred to our hospital for the evaluation of hypergammaglobulinemia (IgG 2898 mg/dL and IgA 587 mg/dL), inflammation (CRP 6.7 mg/dL and serum interleukin-6 (IL-6) 15.1 ng/L), and anemia (Hb 10.9 mg/dL). Castleman's disease (CD) was diagnosed by axillary lymph node biopsy. Five months later, painful purpura (multiple palpable 5 mm lesions) developed on his legs, gradually spreading to the upper limbs, thighs, and trunk, accompanied by arthralgia of the wrists, ankles, and knees. Skin biopsy revealed leukocytoclastic vasculitis with IgA deposits in dermal vessels. Accordingly, IgA vasculitis (Henoch-Schönlein purpura) was diagnosed. Tocilizumab (an anti-IL-6 receptor antibody) was administered intravenously at 8 mg/kg and treatment was repeated at monthly intervals. His purpura and clinical findings specific to CD improved rapidly. CD is well known to cause various skin lesions. The findings in this case indicate that overproduction of IL-6 contributes to IgA vasculitis (Henoch-Schönlein purpura) as well as to the pathogenesis of CD.
Nephrology Center, Toranomon Hospital, 2-2-2, Toranomon, Minato, Tokyo, 105-8470, Japan. yoichi-o-s@hotmail.co.jp.
Yoichi
http://orcid.org/0000-0003-1908-6329
Y
Oshima
Nephrology Center, Toranomon Hospital, 2-2-2, Toranomon, Minato, Tokyo, 105-8470, Japan.
Junichi
J
Hoshino
Nephrology Center, Toranomon Hospital, 2-2-2, Toranomon, Minato, Tokyo, 105-8470, Japan.
Tatsuya
T
Suwabe
Nephrology Center, Toranomon Hospital, 2-2-2, Toranomon, Minato, Tokyo, 105-8470, Japan.
Noriko
N
Hayami
Nephrology Center, Toranomon Hospital, 2-2-2, Toranomon, Minato, Tokyo, 105-8470, Japan.
Masayuki
M
Yamanouchi
Nephrology Center, Toranomon Hospital, 2-2-2, Toranomon, Minato, Tokyo, 105-8470, Japan.
Akinari
A
Sekine
Nephrology Center, Toranomon Hospital, 2-2-2, Toranomon, Minato, Tokyo, 105-8470, Japan.
Toshiharu
T
Ueno
Nephrology Center, Toranomon Hospital, 2-2-2, Toranomon, Minato, Tokyo, 105-8470, Japan.
Hiroki
H
Mizuno
Nephrology Center, Toranomon Hospital, 2-2-2, Toranomon, Minato, Tokyo, 105-8470, Japan.
Junko
J
Yabuuchi
Nephrology Center, Toranomon Hospital, 2-2-2, Toranomon, Minato, Tokyo, 105-8470, Japan.
Aya
A
Imafuku
Nephrology Center, Toranomon Hospital, 2-2-2, Toranomon, Minato, Tokyo, 105-8470, Japan.
Masahiro
M
Kawada
Nephrology Center, Toranomon Hospital, 2-2-2, Toranomon, Minato, Tokyo, 105-8470, Japan.
Rikako
R
Hiramatsu
Nephrology Center, Toranomon Hospital, 2-2-2, Toranomon, Minato, Tokyo, 105-8470, Japan.
Eiko
E
Hasegawa
Nephrology Center, Toranomon Hospital, 2-2-2, Toranomon, Minato, Tokyo, 105-8470, Japan.
Naoki
N
Sawa
Nephrology Center, Toranomon Hospital, 2-2-2, Toranomon, Minato, Tokyo, 105-8470, Japan.|Okinaka Memorial Institute for Medical Research, Toranomon Hospital, Tokyo, Japan.
Kenmei
K
Takaichi
Department of Dermatology, Toranomon Hospital, Tokyo, Japan.
Nobukazu
N
Hayashi
Department of Pathology, Toranomon Hospital, Tokyo, Japan.
Takeshi
T
Fujii
Nephrology Center, Toranomon Hospital, 2-2-2, Toranomon, Minato, Tokyo, 105-8470, Japan.|Okinaka Memorial Institute for Medical Research, Toranomon Hospital, Tokyo, Japan.
Yoshifumi
Y
Ubara
D000328:Adult; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use*; D005871:Castleman Disease / Q000150:complications / Q000188:drug therapy*; D006801:Humans; D011695:IgA Vasculitis / Q000150:complications / Q000188:drug therapy*; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D016896:Treatment Outcome
D061067:Antibodies, Monoclonal, Humanized; D007166:Immunosuppressive Agents; C502936:tocilizumab
Castleman’s disease; Henoch-Schönlein purpura; IgA vasculitis; Interleukin-6; Leukocytoclastic vasculitis; Tocilizumab