Felty Syndrome
Original Editors - Students from Bellarmine University's Pathophysiology of Complex Patient Problems project.
Top Contributors - Abby Janszen, Shannon Stenger, Rucha Gadgil, Uchechukwu Chukwuemeka, WikiSysop, Kim Jackson, Claire Knott, Lucinda hampton, 127.0.0.1 and Elaine Lonnemann
Definition/Description[edit | edit source]
Felty Syndrome (FS), a rare complication of rheumatoid arthritis (RA), was first described by Dr. Augustus Felty in 1924. It is characterized by a triad of symptoms: seropositive RA with severe joint involvement, a low white blood cell count (neutropenia), and an enlarged spleen (splenomegaly). However, the triad is not required for diagnosis. Splenomegaly and severe joint involvement are not always present in some people with FS, and some people can even present with no symptoms. Neutropenia is the hallmark symptom of FS and is key for diagnosis. FS can be a potentially life-threatening, serious systemic condition due to its increased risk for infection during an immunosuppressed state of RA.Cite error: Invalid <ref> tag; name cannot be a simple integer. Use a descriptive titleCite error: Invalid <ref> tag; name cannot be a simple integer. Use a descriptive titleCite error: Invalid <ref> tag; name cannot be a simple integer. Use a descriptive title
Prevalence[edit | edit source]
The prevalence of FS is an estimate 10 cases per 100,000 people. It is seen in about 1-3% cases of RA, making it a rare rheumatological disorder. Cite error: Invalid <ref> tag; name cannot be a simple integer. Use a descriptive titleCite error: Invalid <ref> tag; name cannot be a simple integer. Use a descriptive title
FS is three times more common in females than males. However, early occurrence is more common in males. It more commonly affects the Caucasian population, and the mean age of those presenting with FS is between 50-70 years old.Cite error: Invalid <ref> tag; name cannot be a simple integer. Use a descriptive titleCite error: Invalid <ref> tag; name cannot be a simple integer. Use a descriptive title
Characteristics/Clinical Presentation[edit | edit source]
Aside from the classic triad of characteristics of FS, which are RA, neutropenia, and splenomegaly, other signs and symptoms can also present themselves in this rare disorder, as well.Cite error: Invalid <ref> tag; name cannot be a simple integer. Use a descriptive titleCite error: Invalid <ref> tag; name cannot be a simple integer. Use a descriptive title Some of these other signs and symptoms include: Cite error: Invalid <ref> tag; name cannot be a simple integer. Use a descriptive title
- Painful, stiff, swollen joints (hands, feet, arms)
- Fever
- Weight loss
- Fatigue
- Skin discoloration (especially an abnormal brown pigmentation of the leg)
- Ulcers (especially on the lower leg)
- Enlarged liver (hepatomegaly)
- Possible anemia, thrombocytopenia, or vasculitis
It should also be understood that splenomegaly is not always present in FS, and it could indicate other complications (see Associated Co-morbidities).Cite error: Invalid <ref> tag; name cannot be a simple integer. Use a descriptive title
Associated Co-morbidities[edit | edit source]
Despite the associated signs and symptoms accompanying FS, other associated co-morbidities can occur, as well. Infections are common, especially due to neutropenia, and these infections primarily occur at the skin and pulmonary tracts. Nonetheless, neutropenia in FS can also lead to increased risk for cancers, especially Non-Hodgkins Lymphoma. Cite error: Invalid <ref> tag; name cannot be a simple integer. Use a descriptive titleFurthermore, hepatomegaly, lymphadenopathy, episcleritis, sicca syndrome, eyelid necrosis, pleuritic, neuropathy, portal hypertension, liver involvement, such as portal fibrosis or cirrhosis, and anemia can also arise from FS. Cite error: Invalid <ref> tag; name cannot be a simple integer. Use a descriptive title
Additionally, splenomegaly, one of the characteristics of FS, can indicate other complications, such as liver cirrhosis, brucellosis, visceral leishmaniasis, bacterial endocarditis, histoplasmosis, amyloidosis, and abdominal pain. Cite error: Invalid <ref> tag; name cannot be a simple integer. Use a descriptive title
Medications
[edit | edit source]
Immunosuppressive agents and hematopoietic growth factors are two of the most common pharmacological treatments used to reduce mortality and prevent complications in FS. Glucocortoids and antineoplastics/monoclonal antibodies are also used in managing FS.
| Immunosppressive agents | Inhibit factors responsible for immune reactions |
| Methotrexate (Rheumatrex, Trexall) | Antineoplastic agent that is used as a immunosuppressant at lower doses; very effective in treating the RA piece of FS; antirheumatic effects take several weeks to become effective |
| Cyclophosphamide | Antineoplastic alkylating agent and immunosuppressive agent; increases risk of infection by reducing number of B and T cells |
| Hematopoietic growth factor | Stimulate production, maturation, and activation of neutrophils; increases migration and cytotoxicity of neutrophils |
| Filgrastim (Neupogen) | Manages infections that are not responding to antibiotics |
| Sargramostim (Leukine) | Increases granulocytes in 48-91% of patients with FS by stimulating division and maturation of early myeloid and macrophage precursor cells |
| Antineoplastics/Monoclonal Antibodies | Genetically engineered immunoglobulins that work against proteins involved in cell cycle initiation |
| Rituximad (Rituxan) | Genetically engineered antibody (immunoglobulin G1 kappa), which is directed against CD20 antigen of normal and malignant B cells |
Evidence for the Use of Medications in Felty Syndrome
Low dose methotrexate (MTX)
- The most commonly used drug as the initial treatment (4)
- Interferes with interleukin-1 activity and leads to adenosine accumulation (4)
Corticosteroids
- Good initial response for those with FS (4)
- Long term use is a major issue due to increased risk of infection
- Second-line treatment modality (4)
Hydroxychloroquine
- Long lasting effect
- Increased the neutrophil count after a few days (4)
- Supported by a report of 2 cases of FS with severe neutropenia and methotrexate associated toxicity (4)
B-cell depletion therapy with rituximab (4)
- One case report demonstrated sustained remission, but rituximab was ineffective in 2 men with FS in a 6-mo follow up (4)
- Another report with a 46 yo woman with 13 yr history of RA and newly developed FS who both failed to respond to treatment showed that a 12-mo therapy with rituximab resulted in a PMN response (4)
Granulocyte colony-stimulating factor (G-CSF)
- Effective therapeutic modality in the management of severe neutropenia in FS (4)
- Supported by several case reports (4)<br>Some reports of complications developing from this medication (4)
- G-CSF may be associated with flu-like Sx’s, exanthema, thrombocytopenia, hyperuricemia, and increased alkaline phosphatase (4)
- One study of a patient with FS and profound neutropenia associated with recurrent infections showed that G-CSF significantly increased neutrophil count after 24 hrs (4)
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Diagnostic Tests/Lab Tests/Lab Values[edit | edit source]
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Etiology/Causes[edit | edit source]
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Systemic Involvement[edit | edit source]
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Medical Management (current best evidence)[edit | edit source]
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Physical Therapy Management (current best evidence)[edit | edit source]
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Differential Diagnosis[edit | edit source]
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Case Reports/ Case Studies[edit | edit source]
- Felty's Syndrome with hyperthyroidism: A Case Report and Literature Review (view article in ebscohost)
- A Case of Felty's Syndrome Associated with Skin Ulcers and Extranodal NK/T-cell Lymphoma (view article in ebscohost)
- A Case of Adult-onset Felty Syndrome in Juvenile Rheumatoid Arthritis (view article in ebscohost)
- Surgical Management of Portal Hypertension in Felty's Syndrome: A Case Report and Literature Review (view article in ebscohost)
- Diagnosis of Felty's Syndrome, distinguished from hematology cal neoplasm: A Case Report (view article in ebscohost)
- Felty's Syndrome as an initial presentation of Rheumatoid Arthritis: A Case Report (view artical on Biomed Central)
Resources
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Recent Related Research (from Pubmed)[edit | edit source]
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References[edit | edit source]
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