Felty Syndrome: Difference between revisions
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<div class="noeditbox">Welcome to [[Pathophysiology of Complex Patient Problems|PT 635 Pathophysiology of Complex Patient Problems]] This is a wiki created by and for the students in the School of Physical Therapy at Bellarmine University in Louisville KY. Please do not edit unless you are involved in this project, but please come back in the near future to check out new information!!</div><div class="editorbox"> | | ||
<div class="noeditbox">Welcome to [[Pathophysiology of Complex Patient Problems|PT 635 Pathophysiology of Complex Patient Problems]] This is a wiki created by and for the students in the School of Physical Therapy at Bellarmine University in Louisville KY. Please do not edit unless you are involved in this project, but please come back in the near future to check out new information!!</div><div class="editorbox"> | |||
'''Original Editors '''- [[Pathophysiology of Complex Patient Problems|Students from Bellarmine University's Pathophysiology of Complex Patient Problems project.]] | '''Original Editors '''- [[Pathophysiology of Complex Patient Problems|Students from Bellarmine University's Pathophysiology of Complex Patient Problems project.]] | ||
'''Top Contributors''' - {{Special:Contributors/{{FULLPAGENAME}}}} | '''Top Contributors''' - {{Special:Contributors/{{FULLPAGENAME}}}} | ||
</div> | </div> | ||
== Definition/Description == | == Definition/Description == | ||
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.<ref name="1">RUO-ZHI X, MU-JUN X, ZI-JIE L, RUI-FANG F, DONG-JUN L. Diagnosis of Felty’s syndrome, distinguished from hematological neoplasm: A case report. Oncology Letters [serial on the Internet]. (2014 March), [cited April 2, 2016]; 7(3): 713-716. Available from: Academic Search Complete.</ref><ref name="2">Updated by: Gordon A. Starkebaum a. Felty syndrome: MedlinePlus Medical Encyclopedia [Internet]. Nlm.nih.gov/medlineplus/ency/article/000445.htm</ref><ref name="3">Bagher Owlia M, Newmann K, Akhtari M. Felty’s Syndrome, Insights and Updates. TORJ. 2014; 8(1): 129-136.</ref> | 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.<ref name="1">RUO-ZHI X, MU-JUN X, ZI-JIE L, RUI-FANG F, DONG-JUN L. Diagnosis of Felty’s syndrome, distinguished from hematological neoplasm: A case report. Oncology Letters [serial on the Internet]. (2014 March), [cited April 2, 2016]; 7(3): 713-716. Available from: Academic Search Complete.</ref><ref name="2">Updated by: Gordon A. Starkebaum a. Felty syndrome: MedlinePlus Medical Encyclopedia [Internet]. Nlm.nih.gov/medlineplus/ency/article/000445.htm</ref><ref name="3">Bagher Owlia M, Newmann K, Akhtari M. Felty’s Syndrome, Insights and Updates. TORJ. 2014; 8(1): 129-136.</ref> | ||
== Prevalence == | == Prevalence == | ||
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. <ref name="3" /><ref name="4">Felty Syndrome- NORD (National Organization for Rare Disorders) [Internet]. NORD (National Organization for Rare Disorders). 2016 [cited 2 April 2016]. Available from: http://rarediseases.org/rare-diseases/felty-syndrome/</ref> | 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. <ref name="3" /><ref name="4">Felty Syndrome- NORD (National Organization for Rare Disorders) [Internet]. NORD (National Organization for Rare Disorders). 2016 [cited 2 April 2016]. Available from: http://rarediseases.org/rare-diseases/felty-syndrome/</ref> | ||
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.<ref name="3" /><ref name="4" /> | 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.<ref name="3" /><ref name="4" /> | ||
== Characteristics/Clinical Presentation == | == Characteristics/Clinical Presentation == | ||
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*Possible anemia, thrombocytopenia, or vasculitis | *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).<ref name="3" /> | It should also be understood that splenomegaly is not always present in FS, and it could indicate other complications (see Associated Co-morbidities).<ref name="3" /> | ||
== Associated Co-morbidities == | == Associated Co-morbidities == | ||
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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. <ref name="3" /> | 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. <ref name="3" /> | ||
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== Medications == | == Medications == | ||
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== Diagnostic Tests/Lab Tests/Lab Values == | == Diagnostic Tests/Lab Tests/Lab Values == | ||
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== Etiology/Causes == | == Etiology/Causes == | ||
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== Systemic Involvement == | == Systemic Involvement == | ||
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*Felty's Syndrome as an initial presentation of Rheumatoid Arthritis: A Case Report ([http://casesjournal.biomedcentral.com/articles/10.1186/1757-1626-2-206 view artical on Biomed Central]) | *Felty's Syndrome as an initial presentation of Rheumatoid Arthritis: A Case Report ([http://casesjournal.biomedcentral.com/articles/10.1186/1757-1626-2-206 view artical on Biomed Central]) | ||
== Resources <br> == | == Resources <br> == | ||
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== References == | == References == | ||
see [[Adding References|adding references tutorial]]. | see [[Adding References|adding references tutorial]]. | ||
<references /><br> | <references /><br> | ||
[[Category:Bellarmine_Student_Project]] | [[Category:Bellarmine_Student_Project]] | ||
Revision as of 02:29, 6 April 2016
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, 127.0.0.1, Claire Knott, Lucinda hampton 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]
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Diagnostic Tests/Lab Tests/Lab Values[edit | edit source]
add text here
Etiology/Causes[edit | edit source]
add text here
Systemic Involvement[edit | edit source]
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Medical Management (current best evidence)[edit | edit source]
add text here
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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add appropriate resources here
Recent Related Research (from Pubmed)[edit | edit source]
see tutorial on Adding PubMed Feed
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References[edit | edit source]
see adding references tutorial.
