Small vessel vasculitis and relapsing panniculitis in tumour necrosis factor receptor associated periodic syndrome (TRAPS).
METHODS
A 66 year old female patient had relapsing fever and non-suppurative panniculitis suggestive of enigmatic "Weber-Christian disease" (WCD). Antineutrophil cytoplasmic antibodies with specificity for human leucocyte elastase (HLE-ANCA) were detected. A biopsy showed small vessel vasculitis and panniculitis. A 53 year old man had recurrent episodes of abdominal pain, erythematous rash, and myalgia. Fever attacks had stopped a few years ago. A biopsy showed panniculitis and fasciitis. In both patients mutations (R92Q, T50M) of the tumour necrosis factor receptor super family (TNFRSF) 1A gene were disclosed. Mutations of the TNFRSF 1A gene are the cause of tumour necrosis factor receptor associated periodic syndrome (TRAPS). Both patients responded favourably to treatment with the human soluble p75 TNF alpha receptor fusion protein etanercept (2 x 25 mg subcutaneously/week).


CONCLUSIONS
Small vessel vasculitis and panniculitis have not been reported in TRAPS so far. The cases underline the importance of TNF alpha regulation in inflammatory processes including vasculitis. Genetically determined causes of fever may account for some cases of WCD.
Lamprecht
P
P
Department of Rheumatology, University Hospital of Schleswig-Holstein, 23538 Luebeck, Germany. lamprecht@rheuma-zentrum.de
Moosig
F
F
Adam-Klages
S
S
Mrowietz
U
U
Csernok
E
E
Kirrstetter
M
M
Ahmadi-Simab
K
K
Schroder
J O
JO
Gross
W L
WL
D000368:Aged; D018501:Antirheumatic Agents / Q000627:therapeutic use; D000068800:Etanercept; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D009154:Mutation; D015434:Panniculitis / Q000188:drug therapy / Q000235:genetics* / Q000276:immunology; D010201:Panniculitis, Nodular Nonsuppurative / Q000188:drug therapy / Q000235:genetics* / Q000276:immunology; D018124:Receptors, Tumor Necrosis Factor / Q000627:therapeutic use; D047888:Receptors, Tumor Necrosis Factor, Type I / Q000235:genetics*; D013577:Syndrome; D014657:Vasculitis / Q000188:drug therapy / Q000235:genetics* / Q000276:immunology
D018501:Antirheumatic Agents; D007074:Immunoglobulin G; D018124:Receptors, Tumor Necrosis Factor; D047888:Receptors, Tumor Necrosis Factor, Type I; D000068800:Etanercept