Felty Syndrome
- PMID: 31536299
- Bookshelf ID: NBK546693
Felty Syndrome
Excerpt
Felty syndrome, also known as Chauffard-Still-Felty disease, is an uncommon extra-articular manifestation of seropositive rheumatoid arthritis (RA) characterized by RA, neutropenia (ANC<1500 mm3), and splenomegaly. HLA-DR4 has been noted to be present in >90% of Felty syndrome cases. The American physician Augustus Felty described Felty syndrome in 1924 at Johns Hopkins Hospital. He described 5 unusual cases with common features of chronic arthritis of about 4 years duration, splenomegaly, and striking leukopenia. The term was first used by Hanrahan and Miller in 1932 when they described the beneficial effect of splenectomy in a patient with features similar to the 5 cases reported by Felty. While Felty syndrome characteristically demonstrates chronic arthritis, splenomegaly, and neutropenia; completion of the triad is not necessary for the diagnosis. Neutropenia, however, is a hallmark feature of the disease and cannot be absent. This syndrome poses diagnostic challenges due to its infrequency and variable clinical presentation. Felty syndrome not only warrants astute clinical observation and diagnostic acumen but also demands a multidisciplinary approach to comprehensive management, incorporating the skills and expertise of physicians, advanced care practitioners, nurses, pharmacists, and other health professionals.
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- Campion G, Maddison PJ, Goulding N, James I, Ahern MJ, Watt I, Sansom D. The Felty syndrome: a case-matched study of clinical manifestations and outcome, serologic features, and immunogenetic associations. Medicine (Baltimore) 1990 Mar;69(2):69-80. - PubMed
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- van der Helm-van Mil AH, Verpoort KN, Breedveld FC, Huizinga TW, Toes RE, de Vries RR. The HLA-DRB1 shared epitope alleles are primarily a risk factor for anti-cyclic citrullinated peptide antibodies and are not an independent risk factor for development of rheumatoid arthritis. Arthritis Rheum. 2006 Apr;54(4):1117-21. - PubMed
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