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Published on: February 8, 2019
Coexistence of Takayasu's arteritis with familial Mediterranean fever
Figen Yargucu Zihni1, Melike Kalfa, Pınar Talu Ocakçı
1Division of Rheumatology, Department of Internal Medicine, Ege University School of Medicine, 80. Sk. No: 27/3, 35040, Bornova, Izmir, Turkey.
Abstract:
Familial Mediterranean fever (FMF) is the most common autoinflammatory disease characterized by recurrent self-limited attacks of fever, accompanied with peritonitis, pleuritis or arthritis. It is well known that FMF may coexist with vasculitic pathologies, especially with those involving small and medium vessels. Among the vasculitic pathologies reported to be associated with FMF, Henoch-Schönlein purpura and polyarteritis nodosa come the first, possibly followed up by protracted febrile myalgia. However, coexistence of FMF with any large vessel vasculitis has not been reported to date. Here, we present a case with FMF who later developed Takayasu arteritis, with a severe disease course, being resistant to corticosteroids and conventional immunosuppressive agents, and requiring infliximab treatment.
Insights
Familial Mediterranean fever (FMF), an autoinflammatory disease, can rarely coexist with large vessel vasculitis. This case report details an FMF patient who developed severe Takayasu arteritis, resistant to standard treatments.
Area of Science:
- Rheumatology
- Immunology
- Internal Medicine
Background:
- Familial Mediterranean fever (FMF) is the most prevalent autoinflammatory disorder, marked by recurrent fever and serositis.
- FMF is known to associate with small and medium vessel vasculitides like Henoch-Schönlein purpura and polyarteritis nodosa.
- Coexistence of FMF with large vessel vasculitis has not been previously documented.
Observation:
- This report describes a patient diagnosed with FMF who subsequently developed Takayasu arteritis.
- The patient experienced a severe disease course of Takayasu arteritis.
- The condition proved resistant to corticosteroids and conventional immunosuppressive therapies.
Findings:
- The patient with FMF and Takayasu arteritis required treatment with infliximab due to disease severity and resistance to other therapies.
- This case highlights a novel association between FMF and large vessel vasculitis.
Implications:
- This finding expands the spectrum of vasculitic pathologies associated with FMF.
- It suggests the need for heightened vigilance for large vessel involvement in FMF patients, particularly those with refractory disease.
- Further research may elucidate sharedPathogenic mechanisms between FMF and large vessel vasculitis.
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