U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Felty syndrome

MedGen UID:
4674
•Concept ID:
C0015773
•
Disease or Syndrome
Synonyms: Familial Felty's syndrome; Felty's syndrome; Rheumatoid arthritis, splenomegaly and neutropenia
SNOMED CT: Felty syndrome (57160007); Felty's syndrome (57160007); Rheumatoid arthritis, leukopenia AND splenomegaly (57160007)
Modes of inheritance:
Unknown inheritance
MedGen UID:
989040
•Concept ID:
CN307042
•
Finding
Source: Orphanet
Hereditary clinical entity whose mode of inheritance is unknown.
See: This record
Unknown inheritance (Orphanet)
 
Monarch Initiative: MONDO:0007603
OMIM®: 134750
Orphanet: ORPHA47612

Definition

Felty syndrome (FS), also known as ''super rheumatoid'' disease, is a severe form of rheumatoid arthritis (RA), characterized by a triad of RA, splenomegaly and neutropenia, resulting in susceptibility to bacterial infections. [from ORDO]

Clinical features

From HPO
Rheumatoid arthritis
MedGen UID:
2078
•Concept ID:
C0003873
•
Disease or Syndrome
Rheumatoid arthritis is an inflammatory disease, primarily of the joints, with autoimmune features and a complex genetic component.
See: Feature record | Search on this feature
Splenomegaly
MedGen UID:
52469
•Concept ID:
C0038002
•
Finding
Abnormal increased size of the spleen.
See: Feature record | Search on this feature
Neutropenia
MedGen UID:
163121
•Concept ID:
C0853697
•
Finding
An abnormally low number of neutrophils in the peripheral blood.
See: Feature record | Search on this feature
Show allHide all

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVFelty syndrome
Follow this link to review classifications for Felty syndrome in Orphanet.

Professional guidelines

PubMed

Rheumatoid Arthritis: A Brief Overview of the Treatment.
Bullock J, Rizvi SAA, Saleh AM, Ahmed SS, Do DP, Ansari RA, Ahmed J
Med Princ Pract 2018;27(6):501-507. Epub 2018 Sep 2 doi: 10.1159/000493390. PMID: 30173215Free PMC Article
Management of neutropenia in patients with rheumatoid arthritis.
Lazaro E, Morel J
Joint Bone Spine 2015 Jul;82(4):235-9. Epub 2015 Mar 26 doi: 10.1016/j.jbspin.2015.01.005. PMID: 25819216
Current concepts and treatment for the rheumatoid wrist.
Rizzo M, Cooney WP 3rd
Hand Clin 2011 Feb;27(1):57-72. doi: 10.1016/j.hcl.2010.09.004. PMID: 21176801

Recent clinical studies

Etiology

Rheumatoid Arthritis: A Brief Overview of the Treatment.
Bullock J, Rizvi SAA, Saleh AM, Ahmed SS, Do DP, Ansari RA, Ahmed J
Med Princ Pract 2018;27(6):501-507. Epub 2018 Sep 2 doi: 10.1159/000493390. PMID: 30173215Free PMC Article
Management of neutropenia in patients with rheumatoid arthritis.
Lazaro E, Morel J
Joint Bone Spine 2015 Jul;82(4):235-9. Epub 2015 Mar 26 doi: 10.1016/j.jbspin.2015.01.005. PMID: 25819216
Treatment of pseudo Felty's syndrome: Is there a place for rituximab?
Verhoeven F, Guillot X, Prati C, Wendling D
Joint Bone Spine 2015 May;82(3):196-9. Epub 2015 Jan 23 doi: 10.1016/j.jbspin.2014.12.001. PMID: 25623522
Management of autoimmune neutropenia in Felty's syndrome and systemic lupus erythematosus.
Newman KA, Akhtari M
Autoimmun Rev 2011 May;10(7):432-7. Epub 2011 Jan 18 doi: 10.1016/j.autrev.2011.01.006. PMID: 21255689
Rheumatoid arthritis: relationship with HLA-D.
Stastny P
Am J Med 1983 Dec 30;75(6A):9-15. doi: 10.1016/0002-9343(83)90470-9. PMID: 6362407

Diagnosis

Felty syndrome: a case report.
Gupta A, Abrahimi A, Patel A
J Med Case Rep 2021 May 27;15(1):273. doi: 10.1186/s13256-021-02802-9. PMID: 34039422Free PMC Article
Skin manifestations of rheumatoid arthritis.
Lora V, Cerroni L, Cota C
G Ital Dermatol Venereol 2018 Apr;153(2):243-255. Epub 2018 Jan 24 doi: 10.23736/S0392-0488.18.05872-8. PMID: 29368864
Treatment of pseudo Felty's syndrome: Is there a place for rituximab?
Verhoeven F, Guillot X, Prati C, Wendling D
Joint Bone Spine 2015 May;82(3):196-9. Epub 2015 Jan 23 doi: 10.1016/j.jbspin.2014.12.001. PMID: 25623522
Rheumatoid arthritis: a review of the cutaneous manifestations.
Sayah A, English JC 3rd
J Am Acad Dermatol 2005 Aug;53(2):191-209; quiz 210-2. doi: 10.1016/j.jaad.2004.07.023. PMID: 16021111
Congenital and acquired neutropenia.
Berliner N, Horwitz M, Loughran TP Jr
Hematology Am Soc Hematol Educ Program 2004:63-79. doi: 10.1182/asheducation-2004.1.63. PMID: 15561677

Therapy

Tocilizumab treatment in Felty's syndrome.
Li R, Wan Q, Chen P, Mao S, Wang Q, Li X, Yang Y, Dong L
Rheumatol Int 2020 Jul;40(7):1143-1149. Epub 2020 Apr 28 doi: 10.1007/s00296-020-04588-3. PMID: 32347340
Rheumatoid Arthritis: A Brief Overview of the Treatment.
Bullock J, Rizvi SAA, Saleh AM, Ahmed SS, Do DP, Ansari RA, Ahmed J
Med Princ Pract 2018;27(6):501-507. Epub 2018 Sep 2 doi: 10.1159/000493390. PMID: 30173215Free PMC Article
Skin manifestations of rheumatoid arthritis.
Lora V, Cerroni L, Cota C
G Ital Dermatol Venereol 2018 Apr;153(2):243-255. Epub 2018 Jan 24 doi: 10.23736/S0392-0488.18.05872-8. PMID: 29368864
Treatment of pseudo Felty's syndrome: Is there a place for rituximab?
Verhoeven F, Guillot X, Prati C, Wendling D
Joint Bone Spine 2015 May;82(3):196-9. Epub 2015 Jan 23 doi: 10.1016/j.jbspin.2014.12.001. PMID: 25623522
Felty's syndrome.
Balint GP, Balint PV
Best Pract Res Clin Rheumatol 2004 Oct;18(5):631-45. doi: 10.1016/j.berh.2004.05.002. PMID: 15454123

Prognosis

Felty syndrome: a case report.
Gupta A, Abrahimi A, Patel A
J Med Case Rep 2021 May 27;15(1):273. doi: 10.1186/s13256-021-02802-9. PMID: 34039422Free PMC Article
Porto-Sinusoidal Vascular Disease as the Cause of Portal Hypertension in Felty's Syndrome: A Case Report and Literature Review.
Yang S, Quan M, Li Y, Pan CQ, Xing H
Biomed Res Int 2020;2020:2618260. Epub 2020 Jul 1 doi: 10.1155/2020/2618260. PMID: 32714976Free PMC Article
Treatment of pseudo Felty's syndrome: Is there a place for rituximab?
Verhoeven F, Guillot X, Prati C, Wendling D
Joint Bone Spine 2015 May;82(3):196-9. Epub 2015 Jan 23 doi: 10.1016/j.jbspin.2014.12.001. PMID: 25623522
Felty's syndrome without rheumatoid arthritis?
Rozin A, Hoffman R, Hayek T, Balbir-Gurman A
Clin Rheumatol 2013 May;32(5):701-4. Epub 2013 Jan 5 doi: 10.1007/s10067-012-2157-3. PMID: 23292482
Congenital and acquired neutropenia.
Berliner N, Horwitz M, Loughran TP Jr
Hematology Am Soc Hematol Educ Program 2004:63-79. doi: 10.1182/asheducation-2004.1.63. PMID: 15561677

Clinical prediction guides

Neutrophil activation and B-cell stimulation in the pathogenesis of Felty's syndrome.
Dwivedi N, Radic M
Pol Arch Med Wewn 2012;122(7-8):374-9. PMID: 22814518
The spectrum of large granular lymphocyte leukemia and Felty's syndrome.
Liu X, Loughran TP Jr
Curr Opin Hematol 2011 Jul;18(4):254-9. doi: 10.1097/MOH.0b013e32834760fb. PMID: 21546829Free PMC Article
Congenital and acquired neutropenia.
Berliner N, Horwitz M, Loughran TP Jr
Hematology Am Soc Hematol Educ Program 2004:63-79. doi: 10.1182/asheducation-2004.1.63. PMID: 15561677
Rheumatoid arthritis: relationship with HLA-D.
Stastny P
Am J Med 1983 Dec 30;75(6A):9-15. doi: 10.1016/0002-9343(83)90470-9. PMID: 6362407
The hematopoietic effects of lithium.
Boggs DR, Joyce RA
Semin Hematol 1983 Apr;20(2):129-38. PMID: 6348956

Recent systematic reviews

Biological agents in the management of Felty's syndrome: a systematic review.
Narváez J, Domingo-Domenech E, Gómez-Vaquero C, López-Vives L, Estrada P, Aparicio M, Martín-Esteve I, Nolla JM
Semin Arthritis Rheum 2012 Apr;41(5):658-68. Epub 2011 Nov 25 doi: 10.1016/j.semarthrit.2011.08.008. PMID: 22119104

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Reviews

    Related information

    • MeSH
      Related Medical Subject Headings
    • OMIM
      Related records in OMIM
    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed
    • PubMed (OMIM)
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...