MCID: FLT001
MIFTS: 54

Felty's Syndrome malady

Bone, Blood, Genetic categories

Summaries for Felty's Syndrome

Sources:
8Disease Ontology, 43NIH Rare Diseases, 64Wikipedia, 47OMIM, 33MalaCards
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NIH Rare Diseases:43 Felty's syndrome is a rare, potentially serious disorder that is defined by the presence of three conditions: rheumatoid arthritis (ra), an enlarged spleen (splenomegaly) and a decreased white blood cell count (neutropenia), which causes repeated infections. although some individuals with felty's syndrome are asymptomatic, others can develop serious and life-threatening infections. symptoms of felty's syndrome, in addition to those associated with the three conditions stated above, may include fatigue, fever, weight loss, discoloration of patches of skin, mild hepatomegaly (enlarged liver), lymphadenopathy (swelling of lymph nodes), sjögren syndrome, vasculitis, lower-extremity ulcers, and other findings. the exact cause is unknown, but several risk factors have been proposed, including autoimmunity. a few familial cases of the condition have been reported. treatment typically focuses on controlling the underlying ra; immunosuppressive therapy for ra may improve neutropenia and splenomegaly. last updated: 1/6/2011

MalaCards: Felty's Syndrome, also known as felty syndrome, is related to arthritis and juvenile rheumatoid arthritis, and has symptoms including joint/articular deformation, early death/lethality and hepatomegaly/liver enlargement (excluding storage disease). An important gene associated with Felty's Syndrome is C4A (complement component 4A (Rodgers blood group)), and among its related pathways are Rho Family GTPases and Cytokines and Inflammatory Response. The compounds gm-csf and hydrocortisone have been mentioned in the context of this disorder. Affiliated tissues include skin, liver and lymph node, and related mouse phenotypes are immune system and hematopoietic system.

Disease Ontology:8 A syndrome that results in rheumatoid arthritis, splenomegaly and neutropenia.

Wikipedia:64 Felty\'s syndrome, also called Felty syndrome, is characterized by the combination of rheumatoid... more...

Description from OMIM:47 134750

Aliases & Classifications for Felty's Syndrome

Sources:
8Disease Ontology, 43NIH Rare Diseases, 61UMLS, 49Orphanet, 47OMIM, 10DISEASES, 45Novoseek, 57SNOMED-CT, 40NCIt, 27ICD9CM, 35MeSH, 36MESH via Orphanet, 26ICD10 via Orphanet, 58SNOMED-CT via Orphanet, 62UMLS via Orphanet, 25ICD10
See all sources

Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Genetic
Anatomical: Bone, Blood


Characteristics (Orphanet epidemiological data):

49
felty syndrome:
Age of onset: Adulthood; Age of death: Elderly


Aliases & Descriptions:

felty's syndrome 8 43 61
felty syndrome 43 49 47
familial felty's syndrome 43 61
feltys syndrome 10 45
rheumatoid arthritis with splenoadenomegaly and leukopenia 8
splenomegaly-neutropenia-rheumatoid arthritis syndrome 49
rheumatoid arthritis, splenomegaly and neutropenia 43


External Ids:

Disease Ontology8 DOID:11042
NCIt40 C84712
ICD9CM27 714.1
MeSH35 D005258
OMIM47 134750
SNOMED-CT57 156482001, 57160007
MESH via Orphanet36 D005258
ICD10 via Orphanet26 M05.0
SNOMED-CT via Orphanet58 57160007
UMLS via Orphanet62 C0015773
ICD1025 M05.0

Related Diseases for Felty's Syndrome

Sources:
17GeneCards, 18GeneDecks
See all sources

Diseases related to Felty's Syndrome via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 100)
idRelated DiseaseScoreTop Affiliating Genes
1arthritis30.2IL8, IL10, CSF2
2juvenile rheumatoid arthritis10.2
3n syndrome10.2
4large granular lymphocyte leukemia10.2
5natural killer cell leukemia10.0
6adult syndrome10.0
7t-cell large granular lymphocyte leukemia10.0
8t-cell leukemia10.0
9hepatitis10.0EEF1A1
10capillary leak syndrome10.0CSF2
11prostate cancer10.0EEF1A1
12lupus erythematosus10.0C4A
13multiple myeloma10.0CSF3
14hypereosinophilic syndrome10.0CSF2
15glycogen storage disease10.0CSF3
16dermatitis10.0CSF2
17severe combined immunodeficiency10.0IL3
18sarcoma10.0B3GAT1
19human immunodeficiency virus infectious disease10.0B3GAT1
20astrocytoma10.0B3GAT1
21kidney cancer10.0CSF2
22polycythemia vera10.0IL3
23polycythemia10.0IL3
24periodontitis10.0IL8
25bronchiolitis10.0IL8
26rhinitis10.0IL8
27pulmonary fibrosis10.0IL8
28pulmonary eosinophilia10.0IL8
29cystic fibrosis10.0IL8
30gastritis10.0IL8
31chorioamnionitis10.0IL8
32meningitis10.0IL8
33stomatitis10.0CSF2, CSF3
34endometriosis10.0IL8
35malaria10.0IL10
36lung cancer10.0CSF3, CSF2
37hypersensitivity reaction type ii disease10.0IL10
38pulmonary alveolar proteinosis10.0CSF2, IL3
39hepatitis c10.0IL10
40atopy10.0IL10
41myelofibrosis10.0IL3, CSF3
42galactosemia10.0IL10
43common variable immune deficiency10.0IL10
44chronic myelomonocytic leukemia10.0CSF3, CSF2
45pancreatic cancer10.0IL8
46germ cell cancer10.0B3GAT1
47megakaryocytic leukemia10.0IL3, CSF2
48tuberculosis10.0EEF1A1, IL10
49monocytic leukemia10.0IL8, CSF2
50factor vii deficiency10.0CSF3, CSF2

Graphical network of the top 20 diseases related to Felty's Syndrome:



Diseases related to felty's syndrome

Clinical Features for Felty's Syndrome

Sources:
47OMIM, 49Orphanet
See all sources

Clinical features from OMIM:

134750

Clinical synopsis from OMIM:

134750

Symptoms:

49 (show all 35)
  • joint/articular deformation
  • early death/lethality
  • hepatomegaly/liver enlargement (excluding storage disease)
  • splenomegaly
  • chronic/relapsing otitis
  • repeat respiratory infections
  • arthritis/synovitis/synovial proliferation
  • restricted joint mobility/joint stiffness/ankylosis
  • articular/joint pain/arthralgia
  • subcutaneous nodules/lipomas/tumefaction/swelling
  • lung fibrosis
  • immunodeficiency/increased susceptibility to infections/recurrent infections
  • anaemia
  • chronic skin infection/ulcerations/ulcers/cancrum
  • irregular/in bands/reticular skin hyperpigmentation
  • peripheral neuropathy
  • weight loss/loss of appetite/break in weight curve/general health alteration
  • nasal congestion/sinusitis/rhinitis/rhinorrhea
  • autoimmunity/autoimmune reaction/autoantibodies
  • pericardium anomalies/pericarditis/absence/cysts/pericardial effusion
  • recurrent urinary infections
  • osteolysis/osteoclasia/bone destruction/erosions
  • abnormal pleura/hydrothorax/pleuresia/pleural effusion/chylothorax
  • thrombocytopenia/thrombopenia
  • pharyngitis
  • chronic uveitis/blepharitis/episcleritis/scleritis/conjonctivitis/keratitis
  • polynuclear cells/neutrophils anomalies/neutropenia
  • lymphoma
  • sepsis severe/septicemia
  • diffuse/generalised skin hyperpigmentation/melanoderma
  • lymphadenopathy/polyadenopathies
  • ground-glass/reticular/alveolar/interstitial lung/pulmonary opacity
  • cellulitis/panniculitis/pseudocellulitis/inflammation of subcutaneous tissue
  • bone marrow failure/pancytopenia
  • lymphocytes anomalies

Drugs & Therapeutics for Felty's Syndrome

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials, 61UMLS, 41NDF-RT
See all sources

Approved drugs:

Search CenterWatch for Felty's Syndrome

Drug clinical trials:

Search ClinicalTrials for Felty's Syndrome

Search NIH Clinical Center for Felty's Syndrome

Search CenterWatch for Felty's Syndrome

Genetic Tests for Felty's Syndrome

Anatomical Context for Felty's Syndrome

Sources:
33MalaCards
See all sources

MalaCards organs/tissues related to Felty's Syndrome:

33
Skin, Liver, Lymph node, Lung, Spleen, Bone marrow, Myeloid, T cells, B cells, Endothelial

Animal Models for Felty's Syndrome or affiliated genes

Sources:
37MGI, 28inGenious Targeting Laboratory
See all sources

MGI Mouse Phenotypes related to Felty's Syndrome:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053877.3CSF3, IL3, IL10, IL8, EEF1A1, CSF2
2MP:00053977.0EEF1A1, IL8, IL10, IL3, CSF3, CSF2

Publications for Felty's Syndrome

Sources:
51PubMed
See all sources

Articles related to Felty's Syndrome:

(show top 50)    (show all 341)
idTitleAuthorsYear
1
Felty's syndrome autoantibodies bind to deiminated histones and neutrophil extracellular chromatin traps. (22034172)
2012
2
Neutropenia in Felty's syndrome successfully treated with hydroxychloroquine. (17726769)
2007
3
A case of adult-onset Felty syndrome in juvenile rheumatoid arthritis. (16118895)
2005
4
Case report: Response in proteinuria due to AA amyloidosis but not Felty's syndrome in a patient with rheumatoid arthritis treated with TNF-alpha blockade. (15103032)
2004
5
Neutrophil phagocytosis in Felty's syndrome. (11705439)
2001
6
Generalized morphoea in a patient with Felty's syndrome. (11032063)
2000
7
Pulmonary veno-occlusive disease complicating Felty's syndrome. (9893784)
1998
8
Treatment of Felty's syndrome with the haemopoietic growth factor granulocyte colony-stimulating factor (G-CSF). (9519212)
1998
9
Methotrexate treatment in Felty's syndrome. (9734684)
1998
10
T cell receptor alpha-chain and beta-chain junctional region homology in clonal CD3+, CD8+ T lymphocyte expansions in Felty's syndrome. (9125242)
1997
11
Bronchiectasis and Felty's syndrome. (8736662)
1996
12
Successful treatment of agranulocytosis and sepsis with granulocyte colony stimulating factor in a case of Felty's syndrome. (8523369)
1995
13
Granulocyte colony-stimulating factor induction of improved leukocytopenia with inflammatory flare in a Felty's syndrome patient. (7510482)
1994
14
Felty's syndrome and hairy cell leukaemia. (8173860)
1994
15
Oral methotrexate in the treatment of Felty's syndrome. (8478888)
1993
16
GM-CSF versus G-CSF in the treatment of infectious complication in Felty's syndrome--a case report. (1374651)
1992
17
Strong primary selection for the Dw4 subtype of DR4 accounts for the HLA-DQw7 association with Felty's syndrome. (1685490)
1991
18
Methotrexate treatment of Felty's syndrome. (2322031)
1990
19
Correction of granulocytopenia in Felty's syndrome by granulocyte-macrophage colony-stimulating factor. Simultaneous induction of interleukin-6 release and flare-up of the arthritis. (2510837)
1989
20
Complement C4B-null alleles in Felty's syndrome. (3261591)
1988
21
Lack of evidence for the presence of neutrophil autoantibodies in the serum of patients with Felty's syndrome. (3345294)
1988
22
Felty's syndrome in black Americans: a case report. (3594967)
1987
23
Felty's syndrome. (3614181)
1987
24
Simultaneous presentation of rheumatoid arthritis and acute myeloid leukaemia masquerading as Felty's syndrome. (3465419)
1986
25
Radiocolloid scintigraphy in Felty's syndrome. (3487213)
1986
26
Spontaneous pyomyositis in a patient with Felty's syndrome: diagnosis using computerized tomography. (3738584)
1986
27
Immune complexes and the pathogenesis of neutropenia in Felty's syndrome. (3740999)
1986
28
Palindromic rheumatism and Felty's syndrome. (3876082)
1985
29
Felty's syndrome in a child. (6520839)
1984
30
Felty's syndrome associated with high levels of IgA rheumatoid factor. (6742912)
1984
31
Characterization of immune complexes and immunoglobulin G antibodies reactive with neutrophils in the sera of patients with Felty's syndrome. (6772719)
1980
32
Felty's syndrome in blacks. (7362694)
1980
33
Inhibition of human bone marrow-granulocyte precursors by serum from patients with Felty's syndrome. (7190612)
1980
34
Portal hypertension in Felty's syndrome. (7416125)
1980
35
Gastric volvulus and Felty's syndrome. (7362678)
1980
36
Splenectomy for Felty's syndrome. Clinicopathological study of 27 patients. (637640)
1978
37
Noncirrhotic portal hypertension in Felty's syndrome. (299979)
1977
38
Letter: Lithium in Felty's syndrome. (1259276)
1976
39
Polymorphonuclear leukocyte inclusions and impaired bacterial killing in patients with Felty's syndrome. (182887)
1976
40
Retardation of colony growth of in vitro bone marrow culture using sera from patients with Felty's syndrome, disseminated lupus erythematosus (SLE), rheumatoid arthritis, and other disease states. (168905)
1975
41
Felty's syndrome: a rare indication for splenectomy. (1055509)
1975
42
White-cell antibodies and the aetiology of Felty's syndrome. (4546979)
1974
43
Crosby WH:What to treat in Felty's syndrome. (4740616)
1973
44
Cirrhosis of the liver in Felty's syndrome. (4753065)
1973
45
Chemotaxis of polymorphonuclear leukocytes from patients with systemic lupus erythematosus and Felty's syndrome. (4663604)
1972
46
Synovial cyst of ischial tuberosity in Felty's syndrome. (5819664)
1969
47
Splenectomy in Felty's syndrome. (5406368)
1969
48
Associated rheumatoid arthritis, periarteritis nodosa and Felty's syndrome; report of three cases. (13051020)
1953
49
Neutropenia associated with splenomegaly and atrophic arthritis (Felty's syndrome); report of a case in which splenectomy was performed. (15411128)
1950
50
Dysphagia in Felty's syndrome. (15403073)
1950

Genetic Variations for Felty's Syndrome

Expression for genes affiliated with Felty's Syndrome

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
See all sources
Expression patterns in normal tissues for genes affiliated with Felty's Syndrome

Search GEO for disease gene expression data for Felty's Syndrome.

Pathways for genes affiliated with Felty's Syndrome

Sources:
52QIAGEN, 38NCBI BioSystems Database, 53R&D Systems, 30KEGG
See all sources

Pathways related to Felty's Syndrome according to GeneCards/GeneDecks:

(show all 17)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Hide members
10.1CSF2
210.0TRA
39.7IL8, CSF2
4
Hide members
9.6IL10, CSF2
5
Hide members
9.3CSF2, IL3, IL8
69.3IL8, IL3, CSF2
79.2IL8, IL10, C4A
89.2C4A, IL10, IL8
99.2IL8, IL10, CSF2
109.1IL3, IL10, IL8
11
Hide members
9.1CSF2, TRA, IL10
128.9CSF2, CSF3, IL3
13
Cytokine production by Th17 cells in CF (Mouse model)
8.9CSF2, CSF3, IL8
148.4IL10, IL3, CSF3, CSF2
15
Hide members
8.0CSF2, CSF3, IL3, IL10, IL8
16
Hide members
8.0CSF2, CSF3, IL3, IL10, IL8
17
Hide members
8.0CSF2, CSF3, IL3, IL10, IL8

Compounds for genes affiliated with Felty's Syndrome

Sources:
45Novoseek, 29IUPHAR, 2BitterDB, 60Tocris Bioscience, 11DrugBank, 50PharmGKB, 24HMDB
See all sources

Compounds related to Felty's Syndrome according to GeneCards/GeneDecks:

(show top 50)    (show all 118)
idCompoundScoreTop Affiliating Genes
1gm-csf45 2911.4IL10
2hydrocortisone45 2 60 1113.4IL8
3bmmp4510.1CSF2, IL3
4endotoxin459.8C4A, CSF3
5as 10160 4510.6CSF2, IL3, IL10
6lenograstim459.6CSF3, IL3
7romurtide459.6CSF2, CSF3
8poly(i-c)459.6CSF2, IL3, IL8
9azithromycin45 1110.5IL8, IL10, CSF2
10il-12459.5IL8, IL10, CSF2
11imiquimod45 29 60 1112.5IL8, IL10, CSF2
12groalpha459.5IL8, IL10, CSF2
13daniplestim459.4IL3, CSF3
14anaphylatoxin459.4CSF2, C4A, IL3, IL8
15molgramostim459.4CSF2, CSF3, IL3
16pegfilgrastim45 1110.4IL3, CSF3, CSF2
17mesna459.4CSF2, CSF3, IL3
18thiotepa45 50 1111.4CSF2, CSF3, IL3
19ara-ctp459.4CSF2, CSF3, IL3
20mafosfamide459.4CSF2, CSF3, IL3
21rantes459.4CSF2, CSF3, IL3
22ivig459.3CSF2, IL3, IL10
234-hydroperoxycyclophosphamide459.3CSF2, CSF3, IL3
24pixy321459.3IL3, CSF3, CSF2
25amifostine45 1110.3CSF2, CSF3, IL3
26tarc459.3IL8, IL10, CSF2
27idarubicin45 50 1111.3IL3, CSF3, CSF2
28filgrastim45 1110.3IL3, CSF3, CSF2
29quinine45 29 2 1112.3IL10, IL3, CSF2
30mip 1alpha459.2IL8, IL10, IL3, CSF2
31ifosfamide45 1110.2IL3, CSF3, CSF2
32leucovorin45 50 1111.2IL3, CSF3, CSF2
33il 10459.2IL8, IL10, IL3, CSF2
34neopterin459.2IL8, IL10, IL3, CSF2
35mitoxantrone45 50 1111.2CSF2, CSF3, IL3
36methylcellulose459.1CSF2, CSF3, IL3
37pentoxifylline45 1110.1CSF2, IL10, IL8
38ionomycin459.0CSF2, TRA, IL3, IL10
39mycophenolate mofetil45 50 1111.0CSF3, IL3, IL10
40melphalan45 119.9IL3, CSF3, CSF2
41n-formylmethionyl-leucylphenylalanine458.9CSF2, CSF3, IL3, IL8
42ribavirin45 50 1110.8CSF2, CSF3, IL3, IL10
43zidovudine45 119.8IL10, IL3, CSF3, CSF2
44fmlp458.8CSF2, CSF3, IL3, IL8
45hydroxyurea45 50 1110.8CSF2, CSF3, IL3, IL10
46histamine45 29 2410.8CSF2, IL3, IL10, IL8, B3GAT1
47cyclophosphamide45 50 1110.8CSF2, CSF3, IL3, IL10
48ccl345 299.7CSF3, IL10, IL8
49cyclosporin a45 29 6010.3CSF2, CSF3, IL3, IL10, IL8
50dexamethasone45 50 29 1110.7CSF2, CSF3, IL3, IL10, IL8

GO Terms for genes affiliated with Felty's Syndrome

Sources:
16Gene Ontology
See all sources

Cellular components related to Felty's Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1extracellular spaceGO:0056157.6IL8, IL10, IL3, C4A, CSF3, CSF2

Biological processes related to Felty's Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1positive regulation of DNA replicationGO:0457409.8IL3, CSF2
2positive regulation of tyrosine phosphorylation of Stat5 proteinGO:0425239.5IL3, CSF2
3response to molecule of bacterial originGO:0022379.4IL8, IL10
4immune responseGO:0069558.5CSF2, CSF3, IL3, IL8
5cellular response to lipopolysaccharideGO:0712228.4IL8, IL10, CSF3, CSF2

Molecular functions related to Felty's Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1cytokine activityGO:0051258.4IL10, IL3, CSF3, CSF2
2growth factor activityGO:0080838.1IL10, IL3, CSF3, CSF2

Products for genes affiliated with Felty's Syndrome

  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Felty's Syndrome

3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet