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Felty syndromeM05.00

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

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HistoryThis section has been translated automatically.

Felty, 1924

DefinitionThis section has been translated automatically.

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/EpidemiologyThis section has been translated automatically.

In < 1% of patients with chronic polyarthritis.

EtiopathogenesisThis section has been translated automatically.

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

ManifestationThis section has been translated automatically.

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

Clinical featuresThis section has been translated automatically.

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.

LaboratoryThis section has been translated automatically.

Leukocytopenia with relative lymphocytosis, possibly neutropenia.

Rheumatoid factor is strongly positive.

ANA frequently positive

HLA-DR4 positive in 90% of patients.

TherapyThis section has been translated automatically.

S.u. polyarthritis, chronic (rheumatoid arthritis).

LiteratureThis section has been translated automatically.

  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

Authors

Last updated on: 18.09.2022

Author: Prof. Dr. med. Peter Altmeyer