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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
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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
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
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

Diagnosis

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
Felty's syndrome: an analytical review.
Spivak JL
Johns Hopkins Med J 1977 Sep;141(3):156-62. PMID: 330914

Therapy

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
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
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
Inflammatory arthritis and crystal arthropathy: Current concepts of skin and systemic manifestations.
Fazel M, Merola JF, Kurtzman DJB
Clin Dermatol 2018 Jul-Aug;36(4):533-550. Epub 2018 Apr 10 doi: 10.1016/j.clindermatol.2018.04.010. PMID: 30047436
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
Autoimmune manifestations in large granular lymphocyte leukemia.
Bockorny B, Dasanu CA
Clin Lymphoma Myeloma Leuk 2012 Dec;12(6):400-5. Epub 2012 Sep 19 doi: 10.1016/j.clml.2012.06.006. PMID: 22999943
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

Citrullination of autoantigens implicates NETosis in the induction of autoimmunity.
Dwivedi N, Radic M
Ann Rheum Dis 2014 Mar;73(3):483-91. Epub 2013 Nov 29 doi: 10.1136/annrheumdis-2013-203844. PMID: 24291655
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

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

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