Felty’s Syndrome : causes, symptoms, diagnosis, treatment

Felty’s Syndrome is related to three conditions are three conditions that are Neutropenia, Splenomegaly, Rheumatoid arthritis. 

Neutropenia: It is known as reduced levels of neutrophils (white blood cells) in the body.

Splenomegaly: the condition known as the enlarged spleen.

Rheumatoid arthritis: deals with the stiffening and swelling of the joints.

Introduction of Felty’s Syndrome:

Autoimmune Disorder

Felty’s syndrome is known as an autoimmune disorder. Autoimmune disorders happen when antibodies against the invaders start to mistakenly attack the body’s cells and the body’s immune system can’t make a difference between their body cells and pathogens.

Autosomal Dominant Trait

It is also known as autosomal dominance. An autosomal dominant trait is defined as the gene present on the normal or sex chromosome and a single copy of gene-related with disease mutants can cause fatal disease.

Chromosomes have two arms; one is short and referred to as ‘p’ and the long arm is known as ‘q’.

For instance:15p18 means band, 18 is present on the short arm of chromosome 15. The position of genes can be determined by the numbers of bands on chromosomes.

 Signs and symptoms of Felty’s syndrome

  • Weight loss
  • Temperature (fever)
  • Fatigue
  • Discoloration of the skin
  • Painful swollen joints (hands, feet, arms)
  • Sores (ulcers)
  • Lower extremity ulcers
  • Burning eyes
  • Loss of appetite
  • Swelling of lymph nodes
  • Hepatomegaly (abnormal enlargement of liver)
  • Anemia (reduced levels of RBCs)
  • Vasculitis (the infection in the blood vessels)
  • Thrombocytopenia (low levels of blood platelets)

Causes of Felty’s syndrome

The causes of this syndrome are not exactly clear. Clinicians describes the following factors as causes of the Felty’s syndrome:

  • Unknown disturbance in the immunity
  • Gene mutations
  • Allergy
  • Abnormalities in the blood cells
  • Use of immune-suppressing drugs

Rheumatoid Arthritis

characterized by joint deformation, joint stiffening, severe swelling in joints, and bone erosion.

Characteristics of rheumatoid arthritis

  • It is an autosomal disorder.it happens when the immune system weakens and does not work efficiently for defensive purposes.
  • It is also referred to as persistent(chronic) inflammatory disease and affects most, parts and organs of the body such as joints, skin, lungs, blood vessels and heart.

Symptoms of Rheumatoid Arthritis

  • Soft and swollen joints
  • Joint’s rigidity mostly in the morning and during inactivity
  • Weight loss, fever and food aversion
  • Rheumatoid arthritis primarily attacks the joints of fingers and toes or hands and feet as well as the disease progresses arthritis damages the joints of knees, ankles, wrist hips, and shoulders.
  • 40% patients that are suffered with rheumatoid arthritis have deformity and inflammation in the non-joint structures such as:
  • Salivary glands
  • Bone marrow cells
  • Lungs
  • Skin
  • Nerve tissues

 Causes of Rheumatoid arthritis

  1. It occurs when the immune system invades the lining of the membranes that surround your joints known as synovium.
  2. Stiffening of the lining results due to inflammation, which can damage the bone and cartilage in the joint.
  3. The joint deformation occurs when ligaments and tendons that retain the joint together stretch and weaken, and the joint also loses its alignment.

Risk Factors for Rheumatoid Arthritis

Following are the noticeable risk factors of rheumatoid arthritis which damaged the patients mostly.

 Age:

People can be affected by rheumatoid arthritis at any age. but it mostly begins in the people of middle age.

Obesity:

The higher risk of Rheumatoid arthritis develops mostly in younger people and women at age of 55 who are overweight.

Environmental exposures:

The risk of rheumatoid arthritis increases by the environmental exposure of asbestos.

Family history:

The people have a high danger of infecting with rheumatoid arthritis if they have one person infecting with RA in their family history.

Smoking:

Smoking is considered an immortal environmental factor that causes rheumatoid arthritis. It may be due to some genetic or environmental factors.

Smoking can also cause interstitial lungs related rheumatoid arthritis

Diseases and complications

Lung disorders:

With rheumatoid arthritis chances of lung diseases also increased it includes inflammation in the lungs, discoloration of lung tissues, and difficulty in breathing medically it is known as dyspnea.

Osteoporosis:

It can also occur with rheumatoid arthritis. in osteoporosis bones of the patients weaken and have high chances of fracture.

Carpal Tunnel Syndrome:

This syndrome can also develop if rheumatoid arthritis affects the wrists of the patients. In this case, the nerves that communicate to the hands and fingers become nonfunctional.

Dry eyes and mouth disorders:

Rheumatoid arthritis also causes dry eyes and mouth disorders. In such diseases

Heart problems:

Rheumatoid arthritis can also cause heart problems such as inflammation in the sac of the heart that encircle the heart.it also damaged the arteries and block and rigid them.

Disturbed body composition:

People infected with rheumatoid arthritis can also disturb the body composition of patients. They have huge adipose tissue masses and high fats formation.

Splenomegaly

Spleen is defined as:

Spleen is an organ present on the next side of the abdomen next to the stomach beneath the rib cage

Functions of spleen:

Spleen performs a significant role in filtration of old and destroyed RBCs, neutrophils and all blood cells.

Spleen also performs a defensive role by producing white blood cells against the invaders and mutagens that cause diseases.

 Splenomegaly is defined as:

It is referred to as the enlarged growth of the spleen, it starts to filter both abnormal red blood cells and normal ones.

It also decreases the normal no of cells in the blood.no of platelets decreased due to improper function of the spleen.it can also destroy the organs.

Symptoms of Splenomegaly

Following are the symptoms of splenomegaly:

  • Discomfort in the left side of the abdomen and it can also extend to the left side of the shoulder.
  • Easy bleeding
  • Chlorosis
  • Fatigue and fever
  • Satiety feeling occurs with larger spleen

Causes

Following are the causes of splenomegaly:

  • Parasitic inflammations
  • Hematologic cancer
  • Dismantling of RBCs
  • Malaria
  • Syphilis
  • Metabolic diseases
  • Mononucleosis diseases
  • Endocarditis
  • Destruction of blood cells including platelets and red blood cells
  • Blood clotting in the veins

Risk factors

  1. Risk factors that people might face with splenomegaly are listed below:
  2. People who travel in those that are malaria-infected, have greater chances of splenomegaly.
  3. The people who are infected with viral inflammations are also attacked by splenomegaly.
  4. People also easily attacked by splenomegaly which are infected by glucocerebrosidase deficiency

Complications

  • Enlarged spleen produced many complications such as:
  • It can decrease the number of fit and healthy red blood cells, platelets, and WBC’s in the blood. Anemia and heavy bleeding diseases also occur.
  • When the spleen becomes enlarged the spleen may have ruptured easily. The ruptured spleen can cause severe bleeding in the cavity of the abdomen.

Neutropenia

Neutropenia is known as the low levels of WBCs (neutrophils) in the blood. Neutrophils cells are produced in the bone marrow. Typically, WBC’s and particularly neutrophils perform defensive functions.

Classification of Neutropenia:

  1. Congenital Neutropenia: it is a gene associated with neutropenia; patients suffered from birth.
  2. Acute Neutropenia: in this condition, neutropenia lasts for short periods.
  • Acquired Neutropenia: it is a state which occurs over a specific duration of time.
  1. Chronic neutropenia: it may be fatal and remains for long periods.

Neutropenia is categorized in three types on the bases of no of white blood cells in bloodstream:

Mild Neutropenia: The range of white blood cells (neutrophils) is 1000- 1500 in Mild Neutropenia.

Severe Neutropenia: In severe neutropenia, the range of neutrophils is less than 500.

Moderate Neutropenia: The no of white blood cells in the moderate neutropenia ranges from 500-1000.

Signs and symptoms

Mild neutropenia is less harmful to the patients so symptoms are typical.

Some common symptoms are categorized as:

  • Temperature (fever)
  • Repeated infections
  • Ulcers
  • Stiffening and swelling
  • Causes of Neutropenia:
  • Less production of neutrophils
  • Bone marrow diseases
  • Excessive use rather than formation
  • Infection like TB, sepsis, severe hepatitis
  • Immoderate chemotherapies
  • Tumors formation and other cancerous diseases
  • Autoimmune disorders such as RA and lupus.
  • Low levels of minerals and vitamins particularly deficiency of vitamin B12 and copper

Risk factors

Neutropenia can occur at any age to anyone.

It can also cause the result of medications of chemotherapy.

Neutropenia can cause some infections in some patients.

People that are using cancerous medicines have a higher rate to infect with neutropenia.

People infatuated with this disease have 1500 neutrophils in their bloodstream that is considered normal.

Affected populations

  1. People that are infected with rheumatoid arthritis are due to Felty ‘s syndrome in most of the cases.
  2. Most people affected with Felty’s syndrome have an age range between 50 to 70 years.
  3. Generally, women are three times more easily affected with Felty’s syndrome as compared to men.
  4. The people in Africa are less affected by Felty’s syndrome.
  5. Most of the population of the European region are also less infected with Felty’s syndrome.
  6. Falty’s syndrome rarely affects children and more likely affects people with an age of 50s,60s, and 70s.

Diagnosis and treatment of Felty’s syndrome

  • The treatment of Felty’s syndrome is known. This syndrome can be treated more effectively by curing rheumatoid arthritis.
  • After the treatment of RA, the risk of neutropenia and splenomegaly also decreased.
  • It can be treated by several ways:
  • It can be treated by using anti-infectious drugs that are non-steroidal in composition.
  • Felty’s syndrome can be treated by chelating drugs such as Penicillamine. These drugs are used to eliminate harmful elements and materials from the body.
  • Felty’s syndrome including rheumatoid arthritis can be cured by using a balanced diet, proper exercise, morning walks, and complete bed rest.

Magnetic Resonance Imaging:

It is used to diagnose splenomegaly (enlarged spleen), to see the distinct images, and also used in the recognition of swollen joints in rheumatoid arthritis.

MRI performs a crucial role in the treatment of neutropenia to prognoses reduced level of neutrophils and white blood cells in the bloodstream, RI technique is used and it performs crucial role.

Computer Tomography Technique:

CT scans method is also used to treat Felty’s syndrome, it shows very fine and distinct x-rays of different damaged and infected parts from different angles.

Splenectomy:

Splenectomy is the type of surgical treatment in which a damaged spleen is removed from the body. After splenectomy, possibilities of neutropenia disorder and long-lasting (chronic) infections also reduced.

All these drugs are antifolate and Amethopterin. These medicines are used in low quantities.

  • By using antifolate drugs some side effects may occur such as mouth sores, mouth blisters, and nausea. They can also damage the liver.
  • Physicians can also recommend several drugs such as Glucocorticoids drugs, Leflunomide drugs, and Abatacept drugs.
  • Rituximab drug is also used for the proper functioning of the immune system.
  • GCF is used as blood growth factors. Granulocyte stimulating drugs play an important role in the production of white blood cells they also help in fighting against invaders and mutants.

 

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