Felty syndrome
Updates to Article Attributes
Felty syndrome's Syndrome is is a rare autoimmune disorder that is associated with rheumatoid arthritis. It is characterised by the triad of rheumatoid arthritis, splenomegaly (enlargement of the spleen), and neutropenia (a condition in which there is a low number of white blood cells). In this condition, inflammation and damage to the joints and surrounding tissues can occur, leading to pain, swelling, and restricted movement.
Definition: Felty's Syndrome is a type of autoimmune disorder that is associated with rheumatoid arthritis and characterised by the triad of rheumatoid arthritis, splenomegaly, and neutropenia.
Terminology: Felty's Syndrome is also known as Felty's Syndrome or Felty's Disease.
Epidemiology
It is thought to occur in ~ 1% of patients with rheumatoid arthritis 2.
Felty's Syndrome is a rare condition, affecting only a small number of individuals with rheumatoid arthritis. It is more common in women than in men, and it is most commonly diagnosed in people over the age of 50.
Clinical presentation
Felty syndrome comprises of the triad of:
-
typically with severe articular and extra-articular disease
splenomegaly. Splenomegaly, or an enlarged spleen, is also a common finding in Felty's syndrome.
Additionally, patients may have bicytopaenia or pancytopaenia, recurrent bacterial infections, and non-cirrhotic portal hypertension.
Individuals with Felty's Syndrome may experience joint pain, swelling, and restricted movement due to the inflammation and damage to the joints and surrounding tissues. They may also experience fatigue, weakness, weight loss, and an increased risk of infections due to the low number of white blood cells.
They may also have symptoms such as fatigue, weight loss, and anaemia.
Pathology
Pathophysiology:
The exact cause of Felty's syndrome is not known, but it is believed to be related to an abnormal immune response to self-antigens. This results in an abnormal proliferation of cells and chronic inflammation, leading to joint and organ damage.
Gross Anatomical Pathology:
The gross anatomical pathology of Felty's syndrome is characterised by joint and organ damage, specifically in the form of joint inflammation, erosion, and deformities, as well as splenomegaly.
Serological markers
rheumatoid factor (RF): >95% of patients are positive 5
antinuclear antibody (ANA): 47-100% are positive 5
HLA-DR4*0401 antigen: 78% of patients have the antigen 5
large granular lymphocyte (LGL) expansion: ~ 30% of patients may have it 5
Radiographic Features:
Plain radiographs:
individuals with Felty's Syndrome may show signs of joint destruction and osteoporosis (bone loss) due to the inflammation and damage to the joints and surrounding tissues. X-rays may show joint space narrowing, erosions, and deformities.
Ultrasound:
Ultrasound imaging can show changes in the size and shape of the spleen and other organs in individuals with Felty's Syndrome.
Ultrasound exam may show synovial thickening and fluid accumulation within the joints.
CT and HRCT:
CT and HRCT scans can show changes in the lungs and airways in individuals with Felty's Syndrome, including bronchiectasis (a condition in which the airways become widened and distorted), pleural effusions (accumulation of fluid in the space between the lungs and the chest wall), and nodules.
MRI:
MRI scans can show changes in the joints, surrounding tissues, and organs in individuals with Felty's Syndrome.
Treatment and prognosis
Treatment is through immunosuppression such as methotrexate and rituximab 6. If frequent severe bacterial infections are encounted, G-CSF may be administered 6.
History and etymology
It is named after Augustus Roi Felty (1895 -1964), an American physician 1.
Differential diagnosis
-<p><strong>Felty syndrome</strong> is a rare haematological syndrome in <a href="/articles/rheumatoid-arthritis-ra">rheumatoid arthritis</a>.</p><h4>Epidemiology</h4><p>It is thought to occur in ~ 1% of patients with rheumatoid arthritis <sup>2</sup>.</p><h4>Clinical presentation</h4><p>Felty syndrome comprises of the triad of:</p><ul>- +<p><strong>Felty's Syndrome</strong> is a rare autoimmune disorder that is associated with <a href="/articles/rheumatoid-arthritis-ra" title="rheumatoid arthritis">rheumatoid arthritis</a>. It is characterised by the triad of rheumatoid arthritis, splenomegaly (enlargement of the spleen), and neutropenia (a condition in which there is a low number of white blood cells). In this condition, inflammation and damage to the joints and surrounding tissues can occur, leading to pain, swelling, and restricted movement.</p><p><strong>Definition: </strong>Felty's Syndrome is a type of autoimmune disorder that is associated with rheumatoid arthritis and characterised by the triad of rheumatoid arthritis, splenomegaly, and neutropenia.</p><p><strong>Terminology</strong>: Felty's Syndrome is also known as Felty's Syndrome or Felty's Disease.</p><h4>Epidemiology</h4><p>It is thought to occur in ~ 1% of patients with rheumatoid arthritis <sup>2</sup>.</p><p>Felty's Syndrome is a rare condition, affecting only a small number of individuals with rheumatoid arthritis. It is more common in women than in men, and it is most commonly diagnosed in people over the age of 50.</p><h4>Clinical presentation</h4><p>Felty syndrome comprises of the triad of:</p><ul>
-<a href="/articles/rheumatoid-arthritis-ra">rheumatoid arthritis</a><ul><li>-<a href="/articles/rheumatoid-arthritis-ra"></a>typically with severe articular and extra-articular disease</li></ul>- +<p><a href="/articles/rheumatoid-arthritis-ra">rheumatoid arthritis</a></p>
- +<ul><li><p><a href="/articles/rheumatoid-arthritis-ra"></a>typically with severe articular and extra-articular disease</p></li></ul>
-<li><a href="/articles/splenomegaly">splenomegaly</a></li>-<li><a href="/articles/neutropaenia">neutropenia</a></li>-</ul><p>Additionally, patients may have bicytopaenia or pancytopaenia, recurrent bacterial infections, and non-cirrhotic portal hypertension.</p><h4>Pathology</h4><h5>Serological markers</h5><ul>-<li>-<a href="/articles/rheumatoid-factor">rheumatoid factor</a> (RF): >95% of patients are positive<sup> 5</sup>-</li>-<li>-<a href="/articles/antinuclear-antibody">antinuclear antibody</a> (ANA): 47-100% are positive <sup>5</sup>-</li>-<li>HLA-DR4*0401 antigen: 78% of patients have the antigen <sup>5</sup>-</li>-<li>large granular lymphocyte (LGL) expansion: ~ 30% of patients may have it <sup>5</sup>-</li>-</ul><h4>Treatment and prognosis</h4><p>Treatment is through <a href="/articles/immunosuppression">immunosuppression</a> such as methotrexate and rituximab <sup>6</sup>. If frequent severe bacterial infections are encounted, G-CSF may be administered <sup>6</sup>.</p><h4>History and etymology</h4><p>It is named after <strong>Augustus Roi Felty</strong> (1895 -1964), an American physician <sup>1</sup>.</p><h4>Differential diagnosis</h4><ul><li>-<a href="/articles/large-granular-lymphocyte-leukaemia">large granular lymphocyte (LGL) leukaemia</a> <sup>6</sup>-</li></ul>- +<li><p><a href="/articles/splenomegaly">splenomegaly</a>. Splenomegaly, or an enlarged spleen, is also a common finding in Felty's syndrome.</p></li>
- +<li><p><a href="/articles/neutropaenia">neutropenia</a></p></li>
- +</ul><p>Additionally, patients may have bicytopaenia or pancytopaenia, recurrent bacterial infections, and non-cirrhotic portal hypertension.</p><p>Individuals with Felty's Syndrome may experience joint pain, swelling, and restricted movement due to the inflammation and damage to the joints and surrounding tissues. They may also experience fatigue, weakness, weight loss, and an increased risk of infections due to the low number of white blood cells.</p><p>They may also have symptoms such as fatigue, weight loss, and anaemia. </p><h4>Pathology</h4><h6>Pathophysiology: </h6><p>The exact cause of Felty's syndrome is not known, but it is believed to be related to an abnormal immune response to self-antigens. This results in an abnormal proliferation of cells and chronic inflammation, leading to joint and organ damage.</p><h6>Gross Anatomical Pathology: </h6><p>The gross anatomical pathology of Felty's syndrome is characterised by joint and organ damage, specifically in the form of joint inflammation, erosion, and deformities, as well as splenomegaly.</p><h6>Serological markers</h6><ul>
- +<li><p><a href="/articles/rheumatoid-factor">rheumatoid factor</a> (RF): >95% of patients are positive<sup> 5</sup></p></li>
- +<li><p><a href="/articles/antinuclear-antibody">antinuclear antibody</a> (ANA): 47-100% are positive <sup>5</sup></p></li>
- +<li><p>HLA-DR4*0401 antigen: 78% of patients have the antigen <sup>5</sup></p></li>
- +<li><p>large granular lymphocyte (LGL) expansion: ~ 30% of patients may have it <sup>5</sup></p></li>
- +</ul><h5>Radiographic Features: </h5><h6>Plain radiographs:</h6><p> individuals with Felty's Syndrome may show signs of joint destruction and osteoporosis (bone loss) due to the inflammation and damage to the joints and surrounding tissues. X-rays may show joint space narrowing, erosions, and deformities.</p><h6>Ultrasound: </h6><ul>
- +<li><p>Ultrasound imaging can show changes in the size and shape of the spleen and other organs in individuals with Felty's Syndrome.</p></li>
- +<li><p>Ultrasound exam may show synovial thickening and fluid accumulation within the joints.</p></li>
- +</ul><h6>CT and HRCT: </h6><ul><li><p>CT and HRCT scans can show changes in the lungs and airways in individuals with Felty's Syndrome, including bronchiectasis (a condition in which the airways become widened and distorted), pleural effusions (accumulation of fluid in the space between the lungs and the chest wall), and nodules.</p></li></ul><h6>MRI: </h6><ul><li><p>MRI scans can show changes in the joints, surrounding tissues, and organs in individuals with Felty's Syndrome.</p></li></ul><h4>Treatment and prognosis</h4><p>Treatment is through <a href="/articles/immunosuppression">immunosuppression</a> such as methotrexate and rituximab <sup>6</sup>. If frequent severe bacterial infections are encounted, G-CSF may be administered <sup>6</sup>.</p><h4>History and etymology</h4><p>It is named after <strong>Augustus Roi Felty</strong> (1895 -1964), an American physician <sup>1</sup>.</p><h4>Differential diagnosis</h4><ul><li><p><a href="/articles/large-granular-lymphocyte-leukaemia">large granular lymphocyte (LGL) leukaemia</a> <sup>6</sup></p></li></ul>
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