Felty syndrome M05.00

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 18.09.2022

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Synonym(s)

Still-Chauffard-Felty Syndrome; Still-Chauffard Raymond Syndrome

History
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Felty, 1924

Definition
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Special form of chronic polyarthritis (rheumatoid arthritis) in adulthood with a triad of chronic polyarthritis, leukopenia (especially neutropenia) and splenomegaly. S.a.u. adult breastfeeding syndrome; see also Sjögren's syndrome.

Occurrence/Epidemiology
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In < 1% of patients with chronic polyarthritis.

Etiopathogenesis
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Controversial. An association with the DR4 (HLA-DR4) genotype is discussed.

Manifestation
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Usually 50th-70th LJ and associated with > 10 years of prior chronic polyarthritis. Women are affected twice as often as men.

Clinical features
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Integument: Exanthema with smallest to medulla-sized, raised petechiae, later changing into brown-yellow spots.

In more severe courses, lentil- to coin-sized, reddish to blue-red, later hemorrhagic, itchy, possibly urticarial spots appear. Transition to red urticarial papules, vesicles, hemorrhagic blisters.

Possibly therapy-resistant, superinfected ulcerations (especially pretibially localized).

Frequent recurrent bacterial skin infections, especially furunculosis and abscesses.

Extracutaneous manifestations: Corresponding to primary chronic polyarthritis. Also, lymph node swelling, splenomegaly, hypersplenism with consecutive leukopenia, subfebrile temperatures. Increased bacterial infections of the upper respiratory tract.

Frequent evidence of rheumatoid nodules.

Laboratory
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Leukocytopenia with relative lymphocytosis, possibly neutropenia.

Rheumatoid factor is strongly positive.

ANA frequently positive

HLA-DR4 positive in 90% of patients.

Therapy
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Literature
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  1. Bowman SJ (2002) Hematological manifestations of rheumatoid arthritis. Scand J Rheumatol 31: 251-259
  2. Coakley G et al (2000) Major histocompatility complex haplotypic associations in Felty's syndrome and large granular lymphocyte syndrome are secondary to allelic association with HLA-DRB1 *0401 Rheumatology (Oxford) 39: 393-398
  3. Felty AR (1924) Chronic arthritis in the adult, associated with splenomegaly and leucopenia. A report of 5 cases of an unusual clinical syndrome. Bulletin of the Johns Hopkins Hospital (Baltimore) 35: 16
  4. Hellmich B (2002) Autoantibodies against granulocyte colony-stimulating factor in Felty's syndrome and neutropenic systemic lupus erythematosus. Arthritis Rheum 46: 2384-2391
  5. Hellmich B et al (2002) Low Fcgamma receptor III and high granulocyte colony-stimulating factor serum levels correlate with the risk of infection in neutropenia due to Felty's syndrome or systemic lupus erythematosus. At J Med 113: 134-139
  6. Robertson LP (2000) Generalized morphoea in a patient with Felty's syndrome. J Eur Acad Dermatol Venereol 14: 191-193

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Last updated on: 18.09.2022