Felty's syndrome
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Felty's syndrome | |
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Specialty | Rheumatology |
Felty's syndrome (FS), also called Felty syndrome,[1] is a rare autoimmune disease characterized by the triad of rheumatoid arthritis, enlargement of the spleen and low neutrophil count. The condition is more common in those aged 50–70 years, specifically more prevalent in females than males, and more so in Caucasians than those of African descent. It is a deforming disease that causes many complications for the individual.[2][3]
Signs and symptoms
The symptoms of Felty's syndrome are similar to those of rheumatoid arthritis. Affected individuals have painful, stiff, and swollen joints, most commonly in the joints of the hands, feet, and arms. In some affected individuals, Felty's syndrome may develop during a period when the symptoms and physical findings associated with rheumatoid arthritis have subsided or are not present; in this case, Felty's syndrome may remain undiagnosed. In more rare instances, the development of Felty's syndrome may precede the development of the symptoms and physical findings associated with rheumatoid arthritis.[4]
Felty's syndrome is also characterized by an abnormally enlarged
Complications
- Recurrent infection
- Enlargement of the spleen causing too few red blood cells and platelets in the bloodstream
- Enlarged lymph nodes
- Skin hyperpigmentation
- Cutaneous ulceration
Causes
The cause of Felty's syndrome is unknown, but it has been found to be more common in those with chronic rheumatoid arthritis. Some patients share the HLA-DR4 serotype. This syndrome is mostly present in people having extra articular manifestations of rheumatoid arthritis. People with this syndrome are at risk of infection because they have a low white blood cell count.[6]
Mechanism
The underlying pathogenesis of Felty's syndrome is not clear.[citation needed]
Rheumatoid Arthritis
Rheumatoid arthritis is a condition that cannot be cured but symptoms can be treated using certain medications alone or in conjunction. Due to the increased inflammatory response of the body's immune system, this condition can cause a reduction in red and white blood cells.[8]
Neutropenia
In Felty's syndrome, chronic activation of neutrophils progresses to neutropenia and unabated infections.[9] Neutropenia is a decreased concentration of neutrophils in the blood. Neutrophils are the most abundant cells among white blood cells and play an important role in the immune system by destroying bacteria via phagocytosis. Inflammatory chemicals draw neutrophils to the area where they congregate and fight infection. A decrease in the number of neutrophils stimulates an autoimmune response which leads to arthritis. The loss and destruction of neutrophils leading to neutropenia are, therefore, inflammation-driven due to the body's need for an immune response.[9]
Splenomegaly
Diagnosis
This condition affects less than 1% of patients with rheumatoid arthritis.
A: Arthritis (rheumatoid) [16]
Conditions of the blood
A complete blood count (CBC) can be done to diagnose anemia (normochromic, normocytic), thrombocytopenia, and neutropenia.[17] Abnormal liver function tests are commonly used to help in diagnosis as the spleen and liver are strongly affected by one another.[citation needed]
Splenomegaly
If rheumatoid arthritis is present and other symptoms occur that are not commonly found within RA itself, such as a palpable spleen, further testing should be done. A palpable spleen is not always a clinical significance, therefore CT scan, MRI, or ultrasound can be administered in order to help diagnose the condition. According to Poulin et al., dimensional guidelines for diagnosing splenomegaly are as follows:[18]
- Moderate if the largest dimension is 11–20 cm
- Severe if the largest dimension is greater than 20 cm
Rheumatoid arthritis
RA in patients with Felty's syndrome is chronic (after 10–15 years), and presents with increased severity along with extra articular manifestations.[9] RA can be mistaken for other conditions such as gout if not clinically diagnosed. Diagnosis can be confirmed by use of X-rays or synovial fluid analysis.[19]
Treatment
There is no real treatment for Felty's syndrome, rather the best method in management of the disease is to control the underlying rheumatoid arthritis. Immunosuppressive therapy for RA often improves granulocytopenia and splenomegaly; this finding reflects the fact that Felty's syndrome is an immune-mediated disease. A major challenge in treating FS is recurring infection caused by neutropenia. Therefore, in order to decide upon and begin treatment, the cause and relationship of neutropenia with the overall condition must be well understood.[9] Most of the traditional medications used to treat RA have been used in the treatment of Felty's syndrome. No well-conducted, randomized, controlled trials support the use of any single agent. Most reports on treatment regimens involve small numbers of patients.[20]
Splenectomy may improve neutropenia in severe disease.
Use of rituximab[21] and leflunomide[22] have been proposed.
Use of gold therapy has also been described.[23][24]
Prognosis
Prognosis is dependent on the severity of symptoms and the patient's overall health.[citation needed]
Epidemiology
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History
The condition was named after its discoverer, the American physician Augustus Roi Felty (1895–1964), who in 1924 saw a patient with a rare combination of conditions: chronic arthritis, splenomegaly, and leucopenia.[25] His investigation of the medical literature revealed descriptions of four other cases. His findings prompted him to publish a description of the syndrome, which was subsequently named after him.[26][27]
See also
- Caplan's syndrome
- Still's disease or Still-Chauffard-Felty syndrome, the juvenile form
References
- ^ Online Mendelian Inheritance in Man (OMIM): 134750
- PMID 15454123.
- ^ a b Woolston, W 2017, 'Felty's Syndrome: A Qualitative Case Study', MEDSURG Nursing, vol. 26, no. 2, pp. 105-118.
- ^ Shiel WC, Driver CB (19 March 2021). "Felty's Syndrome". MedicineNet. Retrieved 11 August 2021.
- ^ "CIGNA – Felty's Syndrome". Retrieved 2008-11-07.
- ^ "HowStuffWorks "Felty's Syndrome – Medical Dictionary"". Archived from the original on 2011-10-05. Retrieved 2008-11-07.
- ^ "Rheumatoid arthritis - Symptoms and causes - Mayo Clinic". www.mayoclinic.org. Retrieved 2017-12-13.
- ^ "Rheumatoid Arthritis". Nucleus Medical Media. November 4, 2017.
- ^ a b c d Dwivedi, N., & Radic, M. (2012). Neutrophil activation and B-cell stimulation in the pathogenesis of Felty's syndrome. Polskie Archiwum Medycyny Wewnetrznej, 122(7-8), 374-379.
- ^ Radhakrishnan N, Sacher RA, Besa EC (Nov 12, 2017). "Splenomegaly: Etiology". MedScape.
- ^ "Felty's Syndrome Causes, Symptoms, Diagnosis and Treatment by MedicineNet.com". Retrieved 2008-11-07.
- ^ "Splenomegaly". The Lecturio Medical Concept Library. Retrieved 11 August 2021.
- ^ "Anemia: Overview". The Lecturio Medical Concept Library. Retrieved 11 August 2021.
- ^ "Neutropenia". The Lecturio Medical Concept Library. Retrieved 11 August 2021.
- ^ "Thrombocytopenia". The Lecturio Medical Concept Library. Retrieved 11 August 2021.
- ^ "Rheumatoid Arthritis". The Lecturio Medical Concept Library. Retrieved 11 August 2021.
- ^ "Thrombocytopenia: Diagnosis". NHI; National Heart, Lung, and Blood Institute. Nov 12, 2017.
- ^ Neetu Radhakrishnan (2019-10-20). "Splenomegaly". Medscape. Updated Apr. 2012 referring the classification system to Poulin et al.
- PMID 17910294.
- ^ Keating, Richard M. "eMedicine – Felty's Syndrome". Retrieved 2008-11-07.
- S2CID 29359015.
- PMID 11327265.
- ISBN 978-0-7817-7820-6. Retrieved 14 November 2010.
- PMID 15630719.
- ^ Brief biography of Felty: "Augustus Roi Felty", Whonamedit?
- See also: "Felty's syndrome", Whonamedit?
- ^ Felty, A. R. (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.
- .