Rheumatoid Vasculitis

Rheumatoid Vasculitis

Article
Brain & Nerve
Bone, Muscle, & Joint
+9
Contributed byKrish Tangella MD, MBAApr 14, 2020

What are the other Names for this Condition? (Also known as/Synonyms)

  • RV (Rheumatoid Vasculitis)
  • Vasculitis in Rheumatoid Arthritis

What is Rheumatoid Vasculitis? (Definition/Background Information)

  • Rheumatoid Vasculitis (RV) is a form of vasculitis that is observed in the background of severe and long-term rheumatoid arthritis (RA), an autoimmune disorder that mostly affects the lining of joints and surrounding tissues. RV may be described as a rare and severe complication of RA
  • Vasculitis constitute a group of disorders that result in the inflammation of the blood vessels. It can cause damage to various body organs depending on the region affected, due to decreased blood and oxygen supply to the body region. Rheumatoid Vasculitis is a systemic condition that affects the small and medium-sized arteries and veins of the body, due to progression of rheumatoid arthritis
  • Besides the involvement of the joints, such as of the arms, legs, and hips; in Rheumatoid Vasculitis, the skin and nerves are commonly affected. However, any body region/organ, such as the heart, lungs, gastrointestinal tract, kidney, and brain, may be affected
  • The cause of development of Rheumatoid Vasculitis, due to rheumatoid arthritis, is not well understood; but certain factors playing a contributory role include smoking of tobacco, Felty syndrome that is associated with RA, and high levels of rheumatoid factor antibodies in blood
  • A diagnosis of Rheumatoid Vasculitis may involve blood tests, such as C-reactive protein test, erythrocyte sedimentation rate test, and rheumatoid factor blood test, imaging studies, nerve conduction studies, electrocardiogram, endoscopic findings, and tissue biopsies of the skin, muscle, or nerve, towards establishing a definitive diagnosis
  • An early diagnosis and appropriate treatment of Rheumatoid Vasculitis can lead to a good prognosis. The prognosis depends on the severity of the condition, the organs affected, and one’s response to therapy. Recent treatment modalities have decreased the number of cases of Rheumatoid Vasculitis in the last few decades, especially with the advent of newer biologic agents

Who gets Rheumatoid Vasculitis? (Age and Sex Distribution)

  • Rheumatoid Vasculitis is an uncommon condition that is observed in individuals with chronic rheumatoid arthritis, especially middle-aged and older adults
  • Both males and females are affected, and a male predominance is reported
  • It is observed worldwide and can affect people of all racial and ethnic backgrounds

What are the Risk Factors for Rheumatoid Vasculitis? (Predisposing Factors)

The primary risk factor for the onset of Rheumatoid Vasculitis (RV) is severe and chronic rheumatoid arthritis (RA), an inflammatory disorder that mainly affects the joints. It is observed that less than 1 in 100 individuals with RA, which is present for over 10 years or more, may progress to develop RV.

Other factors that influence Rheumatoid Vasculitis development include:

  • High levels of RF antibodies and certain other proteins in blood
  • Presence of Felty syndrome, which is an infrequent disorder of unknown origin that is marked by the presence of three distinct conditions including:
    • Rheumatoid arthritis
    • Decreased neutrophil/granulocyte count in blood, called neutropenia; neutrophils are a type of white blood cells found in blood and bone marrow
    • Enlarged spleen (splenomegaly)
  • Cigarette/tobacco smoking, especially for several years and decades

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Rheumatoid Vasculitis? (Etiology)

  • Rheumatoid Vasculitis (RV) is described as an inflammation of the small and medium-sized blood vessels occurring in the background of chronic rheumatoid arthritis (RA). RV is reportedly similar in nature to other vasculitis forms such as polyarteritis nodosa and cryoglobulinemia
  • The exact mechanism of development of Rheumatoid Vasculitis is not known. It is researched that years of smoking tends to destroy the blood vessels and elevates one’s risk for RV
  • Other factors that accelerate its progression includes elevated blood levels of rheumatoid factor (RF), a type of immunoglobulin associated with RA; and Felty syndrome, which is a triad of conditions that includes RA, very low white blood cell count, and enlarged spleen

In many cases, vasculitis is believed to be the result of a hypersensitivity reaction to certain stimuli triggering an abnormal immune reaction in the body. Such stimuli could be in the form of inflammatory disorders, infections, or various other factors.

What are the Signs and Symptoms of Rheumatoid Vasculitis?

The severity of signs and symptoms of Rheumatoid Vasculitis may vary from one individual to another. In some, it may be mild, resulting in a delayed diagnosis of the condition. In other individuals, it may be severe. The signs and symptoms also depend on the affected organs and systems; any body system may be affected presenting a systemic condition.

The signs and symptoms of underlying rheumatoid arthritis (RA), generally affecting multiple joints, may include:

  • Swelling and tenderness of the joints, decreased range of motion
  • Prolonged stiffness of the joints
  • Firm lumps beneath the skin
  • Joint deformity; often both joints of the arms and legs are affected
  • The small joints, such as the feet and hands, and the larger joints, of the hips, knees, elbows, and shoulders, may be affected
  • Longstanding RA may affect body organs such as the skin, lungs, eye, and heart

The common signs and symptoms (constitutional symptoms) of Rheumatoid Vasculitis include:

  • Fever
  • Fatigue and tiredness
  • Loss of appetite
  • Weight loss
  • Muscle weakness
  • Overall discomfort and malaise
  • Chest/abdomen pain

Typically, the skin is affected followed by any organ(s) of the body,. Based on the progression of Rheumatoid Vasculitis, the following presentations may be noted:

  • Formation of painful red skin rashes
  • The digits of the hands and feet are usually affected due to involvement of the peripheral nerves
  • Fingernails turn red and form sores/ulcers around the nail tips; pitting of the nails is observed
  • Pain, numbness, and tingling sensation may be felt on the hands and feet
  • Inflammation of the eyes results in redness, pain, and impaired vision
  • Severe blood flow obstruction to digits can cause tissue death or necrosis
  • Presence of blood/protein in urine

Based on the involvement of the various organs/systems, the following signs and symptoms may be noted.

Signs and symptoms related to the heart and blood vessels (cardiovascular system):

  • Breathing difficulties
  • Sweating and discomfort
  • Heart palpitations
  • Blood in sputum
  • Confusion
  • Abnormal heartbeats (arrhythmias)
  • Dizziness or lightheadedness
  • Fainting (syncope)

Signs and symptoms related to the lungs and airways (respiratory system):

  • Cough that may be persistent
  • Blood in cough/sputum (hemoptysis)
  • Shortness of breath that gets worse with time
  • Difficulty in breathing
  • Heaviness of chest and chest pain
  • Wheezing, exhibiting a high-pitched sound while breathing (stridor)
  • Changes to one’s voice
  • Hoarseness
  • Loss of voice

Signs and symptoms related to the gastrointestinal (GI) system:

  • Nausea and vomiting
  • Difficulty in swallowing
  • Hoarse voice
  • Indigestion; feeling of fullness after eating a small amount of food
  • Heartburn or acid reflux (gastroesophageal reflux disease or GERD)
  • Abdominal pain and discomfort
  • Abdominal bloating and distension
  • Irregular bowel movements
  • Passing gas; belching or flatus
  • Constipation or diarrhea
  • Unintended loss of weight

Signs and symptoms related to the kidney and urinary bladder (genitourinary system):

  • Unexplained fever
  • Night sweats
  • Difficulty in urinating; presence of burning sensation
  • Feeling of obstruction while urinating
  • Inflammation of the bladder (cystitis)
  • Sharp pain in the back or lower abdomen that radiates to the groin
  • Chronic pain in the pelvis
  • Fluid accumulation in the lower legs (pedal edema)
  • Increased blood pressure

Signs and symptoms related to the brain and spinal cord (central nervous system) and peripheral nervous system (cerebrovascular system):

  • Headaches that may be of sudden onset; persistent headaches
  • Damage to peripheral nerves
  • Loss of strength
  • Neck pain
  • Back pain
  • Visual impairment such as blurred vision, double vision or poor eyesight
  • Hearing loss
  • Insomnia or loss of sleep, or excessive sleepiness (usually during daytime)
  • Tremors
  • Seizures or convulsions
  • Lack of coordination; unsteadiness
  • Vertigo
  • Confusion
  • Fainting
  • Change in behavior
  • Mental impairment
  • Memory loss

It is important to note that the combination of signs and symptoms varies from individual to individual.

How is Rheumatoid Vasculitis Diagnosed?

The diagnosis of Rheumatoid Vasculitis may involve the following tests and examinations:

  • Complete physical examination and a thorough medical history
  • Assessment of the presenting signs and symptoms

Tests and procedures based on the different body part/organ or system that is affected may include any of the following.

  • Blood and urine tests related to overall inflammatory and autoimmune activity:
    • Complete blood count (CBC) with differential: This measures the red blood cell count and hemoglobin levels in blood
    • Erythrocyte sedimentation rate (ESR): ESR is a blood test that can help the healthcare provider identify any inflammatory activity within the body
    • C-reactive protein (CRP) test: This blood test measures the amount of a protein, called C-reactive protein, in blood. It also provides information on the inflammation condition of the body
    • Anti-nuclear antibody (ANA) test: ANA test identifies the antinuclear antibodies within the blood
    • Basic metabolic panel tests
    • Anti-streptolysin O (ASO) titer test, if a streptococcal infection is the suspected trigger
    • Specific antibodies test
    • Protein electrophoresis and immunofixation electrophoresis tests
    • Complement blood test
    • Cryoglobulin blood test
    • HIV antibody blood test
    • Urine tests for protein and presence of blood
    • Urinalysis

Tests and procedures related to the muscles and joints (musculoskeletal system):

  • Laboratory tests, which may include:
    • Creatine kinase test: It is used to measure the amount of creatine kinase in blood. The enzyme creatine kinase is released by the muscle and into the bloodstream, when any deterioration has occurred
    • Rheumatoid factor (RF) blood test
    • Cyclic citrullinated peptide antibody blood test (anti-CCP)
  • Electromyogram (EMG): Electromyographic studies to determine the region of muscle that is affected
  • Radiological imaging studies of the affected region such as:
    • X-ray studies
    • CT scans
    • MRI scans
    • Ultrasound imaging: An ultrasound imaging equipment uses high-frequency sound waves to generate a more detailed image of affected region
    • Musculoskeletal angiographic studies
  • Fine needle aspiration (FNA) of joint fluid: Occasionally, fluid may accumulate around a joint, which results in pain and disability. Analysis of this joint will provide clues regarding the cause of joint fluid accumulation
  • Muscle biopsy: A biopsy of the affected muscle tissue is performed and sent to a laboratory for a pathological examination. A pathologist examines the biopsy sample under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis. Examination of the biopsy sample under a microscope by a pathologist is considered to be gold standard in arriving at a conclusive diagnosis

Tests and procedures related to the skin and underlying tissue (integumentary system):

  • Dermoscopy: It is a diagnostic tool where a dermatologist examines the skin using a special magnified lens
  • Wood’s lamp examination: In this procedure, the healthcare provider examines the skin using ultraviolet light. It is performed to examine the change in skin pigmentation
  • Punch biopsy of skin for immunofluorescence studies, especially direct immunofluorescence studies. The immunofluorescence deposits can be performed with fluorescein-labeled antibodies against IgG, IgM, IgA, and C3
  • Skin biopsy: A skin biopsy is performed and sent to a laboratory for a pathological examination. The pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis

Tests and procedures related to the heart and blood vessels (cardiovascular system):

  • Electrocardiogram (EKG or ECG): It is used to measure the electrical activity of the heart to detect arrhythmias
  • Electrophysiological studies of the heart, to determine where arrhythmia is getting generated in the heart, is often helpful
  • Checking oxygen levels in blood through the skin using a pulse oximeter
  • Checking oxygen levels in blood using an arterial blood gas test
  • Radiological imaging studies that include:
    • CT scan of the thorax
    • MRI scan of the heart
    • Echocardiography: This procedure uses sound waves to create a motion picture of the heart movement. It can help show the size and shape of the heart and how well the chambers and valves are working
    • Doppler ultrasound: Sound waves are used to measure the speed and direction of blood flow
  • Cardiac catheterization: The passage of a thin catheter into the right or left side of the heart to diagnose (and may be treat) cardiovascular conditions
  • Tissue biopsy:
    • A tissue biopsy is performed and sent to a laboratory for a pathological examination. A pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis. Examination of the biopsy under a microscope by a pathologist is considered to be gold standard in arriving at a conclusive diagnosis
    • Biopsy specimens are studied initially using Hematoxylin and Eosin staining. The pathologist then decides on additional studies depending on the clinical situation
    • Sometimes, the pathologist may perform special studies, which may include immunohistochemical stains, molecular testing, and very rarely, electron microscopic studies to assist in the diagnosis

Tests and procedures related to the lungs and airways (respiratory system):

  • Arterial blood gas analysis
  • Pulmonary function test (PFT)
  • Imaging studies that may include:
    • Chest X-ray
    • CT scan of the chest
    • MRI scan of the lungs
    • Pulmonary angiogram
  • Sputum cytology: This procedure involves the collection of mucus (sputum), coughed-up by the patient, which is then examined in a laboratory by a pathologist

A tissue biopsy refers to a medical procedure that involves the removal of cells or tissues, which are then examined by a pathologist. This can help establish a definitive diagnosis. The different biopsy procedures may include the following:

  • Bronchoscopy: During bronchoscopy, a special medical instrument called a bronchoscope is inserted through the nose and into the lungs to collect small tissue samples. These samples are then examined by a pathologist, after the tissues are processed, in an anatomic pathology laboratory
  • Thoracoscopy: During thoracoscopy, a surgical scalpel is used to make very tiny incisions into the chest wall. A medical instrument called a thoracoscope is then inserted into the chest, in order to examine and remove tissue from the chest wall, which are then examined further
  • Thoracotomy: Thoracotomy is a surgical invasive procedure with special medical instruments to open-up the chest. This allows a physician to remove tissue from the chest wall or the surrounding lymph nodes of the lungs. A pathologist will then examine these samples under a microscope after processing the tissue in a laboratory
  • Fine needle aspiration biopsy (FNAB): During fine needle aspiration biopsy, a device called a cannula is used to extract tissue or fluid from the lungs, or surrounding lymph nodes. These are then examined in an anatomic pathology laboratory, in order to determine any signs of abnormality. Nevertheless, FNAB is not a preferred method for the biopsy of lung tissue
  • Autofluorescence bronchoscopy: It is a bronchoscopic procedure in which a bronchoscope is inserted through the nose and into the lungs and measure light from abnormal precancerous tissue. Samples are collected for further examination by a pathologist

Note: Procedures, such as a bronchoscopy, thoracoscopy, thoracotomy, and autofluorescence bronchoscopy, do not always result in a tissue biopsy.

Tissue biopsy from the affected lung:

  • A biopsy of the tissue is performed and sent to a laboratory for a pathological examination. A pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis. Examination of the biopsy under a microscope by a pathologist is considered to be gold standard in arriving at a conclusive diagnosis
  • Biopsy specimens are studied initially using Hematoxylin and Eosin staining. The pathologist then decides on additional studies depending on the clinical situation
  • Sometimes, the pathologist may perform special studies, which may include immunohistochemical stains, molecular testing, flow cytometric analysis and very rarely, electron microscopic studies, to assist in the diagnosis

Tests and procedures related to the gastrointestinal (GI) system:

  • Liver function tests
  • Viral hepatitis panel
  • Imaging studies that may include:
    • X-ray of chest or abdomen
    • Ultrasound studies of the chest or abdomen
    • CT scan of chest or abdomen
    • MRI studies of abdominal organs
    • Gastrointestinal angiographic studies
    • Magnetic resonance cholangiopancreatography (MRCP): It is a special MRI exam of the hepatobiliary and pancreatic systems
  • Upper GI endoscopy: An endoscopic procedure is performed using an instrument called an endoscope, which consists of a thin tube and a camera. Using this technique, the radiologist can have a thorough examination of the insides of the upper GI tract
  • Colonoscopy: A colonoscopy is a test that allows the physician to look at the inner lining of the colon and rectum. A typical colonoscopy involves using a thin, flexible tube (called a colonoscope), with an attached video camera, to view the colon and rectum
  • Exploratory laparoscopy (diagnostic laparoscopy) may be required, if gastrointestinal symptoms are present. In this procedure, the abdomen is examined using a minimally-invasive technique, and a tissue biopsy and tissue for culture obtained
  • Tissue biopsy: A biopsy of the affected region is performed and sent to a laboratory for a pathological examination. A pathologist examines the biopsy sample under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis. Examination of the biopsy sample under a microscope by a pathologist is considered to be gold standard in arriving at a conclusive diagnosis

Tests and procedures related to the kidney and urinary bladder (genitourinary system):

  • Diagnostic tests may include the following:
    • Urinalysis
    • Blood tests including blood urea nitrogen (BUN) and creatinine levels
    • Kidney function tests
  • Radiological studies that may include:
    • Abdominal X-ray
    • CT scan of the abdomen
    • Ultrasound scan of the kidneys
    • MRI scan of the kidney
    • Intravenous pyelogram (IVP): A dye is injected into the blood vessels and the image of kidney structure is obtained
    • Angiography of the abdominal region
  • Ureteroscopy: Endoscopic study of the upper urinary tract using an endoscope inserted through the urethra
  • Cystoscopy: During a cystoscopy, a narrow tube called a cystoscope is inserted to look directly into the bladder. A local anesthetic is usually administered, in order to make the examination more comfortable

Invasive diagnostic procedures such as:

  • Laparoscopy: A special device is inserted through a small hole into the abdomen, to visually examine it. If necessary, a tissue sample is obtained for further analysis. Exploration of the abdomen using a laparoscope is called ‘exploratory laparoscopy’
  • Laparotomy: The abdomen is opened through an incision for examination, and if required, a biopsy sample obtained. Exploration of the abdomen using laparotomy procedure is called ‘exploratory laparotomy’

Although the above modalities can be used to make the initial diagnosis, a tissue biopsy may be required to make a definitive diagnosis to begin treatment. The tissue for diagnosis can be procured in multiple different ways, and they include:

  • Fine needle aspiration (FNA) biopsy: An FNA biopsy may not be helpful, because one may not be able to visualize the different morphological areas of the affected region. Hence, an FNA biopsy as a diagnostic tool has certain limitations, and an open surgical biopsy is preferred
  • Core biopsy of kidney
  • Open biopsy of kidney

A tissue biopsy is performed and sent to a laboratory for a pathological examination. A pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis. Examination of the biopsy under a microscope by a pathologist is considered to be gold standard in arriving at a conclusive diagnosis.

  • Biopsy specimens are studied initially using Hematoxylin and Eosin staining. The pathologist then decides on additional studies depending on the clinical situation.
  • Sometimes, the pathologist may perform special studies, which may include immunohistochemical stains, molecular testing, and very rarely electron microscopic studies to assist in the diagnosis

Tests and procedures related to the brain and spinal cord (central nervous system):

  • Neurological, motor skills, and cognitive assessment: Checking intellectual ability, memory, mental health and function, language skills, judgment and reasoning, coordination and balance, reflexes, sensory perceptions (space, sight, hearing, touch)
  • Electroencephalography (EEG)
  • Electromyography with nerve conductivity tests
  • Imaging studies that may be performed include:
    • X-ray of head and neck and/or vertebral column
    • CT scan of the head and neck region and/or vertebral column
    • MRI scan of the central nervous system (brain and spine)
    • Cerebral angiographic studies
  • Cerebrospinal fluid analysis, where a spinal tap test may be performed
  • Tissue biopsy of the affected blood vessel or brain:
    • A tissue biopsy is performed and sent to a laboratory for a pathological examination. A pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis. Examination of the biopsy under a microscope by a pathologist is considered to be gold standard in arriving at a conclusive diagnosis
    • Biopsy specimens are studied initially using Hematoxylin and Eosin staining. The pathologist then decides on additional studies depending on the clinical situation
    • Sometimes, the pathologist may perform special studies, which may include immunohistochemical stains, molecular testing, and very rarely, electron microscopic studies to assist in the diagnosis

Other diagnostic examinations may be performed based on the affected region/part of the body and the nature of the coexisting condition.

A differential diagnosis may be considered to eliminate the following conditions presenting similar signs and symptoms:

  • Atherosclerosis
  • Cryoglobulinemia
  • Diabetes
  • Polyarteritis nodosa

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Rheumatoid Vasculitis?

The complications of Rheumatoid Vasculitis that develop depend on the underlying rheumatoid arthritis, the organs affected, severity of the signs and symptoms, and one’s response to treatment.

Complications associated with the muscles and joints (musculoskeletal system) may include:

  • Chronic pain and weakness of the joint affecting the quality of life
  • Formation of scar tissue in the muscle and joint tissue
  • Immobilization due to loss of movement
  • Permanent disability

Complications associated with the skin and underlying tissue (integumentary system) may include:

  1. Emotional stress due to cosmetic concerns
  2. Discomfort, irritability of the affected skin
  3. Severe pain and itchiness of skin leading to acute discomfort and irritation, including sleep disruption
  4. The lesions may occasionally develop bacterial or fungal infections
  5. Permanent scarring

Complications associated with the heart and blood vessels (cardiovascular system) may include:

  1. Severe arrhythmias
  2. Normal function of the heart is severely affected
  3. Atherosclerosis
  4. Pericarditis, causing chest pain
  5. Congestive heart failure
  6. Heart attack
  7. Increased risk for thromboembolism (blood clot obstructing a blood vessel)
  8. Chronic high blood pressure in the blood vessels of the lungs

Complications associated with the lungs and airways (respiratory system) may include:

  • Severe chest pain
  • Severe obstruction of the airways causing acute breathing difficulties
  • Pleural effusion (fluid in the chest)
  • Irreversible lung damage
  • Development of pneumothorax (collapsed lung)
  • Respiratory failure
  • Decreased quality of life

Complications associated with the gastrointestinal (GI) system may include:

  • Inability to eat
  • Weight loss and malnutrition
  • GI tract bleeding
  • Perforation of the GI tract
  • Stricture formation of the food pipe
  • Intestinal ischemia or reduced blood supply to bowels

Complications associated with the kidney and urinary bladder (genitourinary system) may include:

  • Severe pain in the back or lower part of the abdomen
  • Obstruction of the urinary bladder
  • Blockage of the urinary tract may lead to kidney damage
  • Hydronephrosis of kidneys (swollen kidneys)
  • Erectile dysfunction
  • Urinary incontinence (involuntary dripping or leakage of urine)
  • Severe cases may rarely result in kidney failure

Complications associated with the brain and spinal cord (central nervous system) and peripheral nervous system (cerebrovascular system) may include:

  • Severe and persistent headaches
  • Reduced performance at school or work
  • Severely affected quality of life due to mental health issues
  • Brain stroke

Treatment complications: Some complications are known to take place with treatment; in some cases, due to treatment also. Immunosuppressive therapy that is used to treat Rheumatoid Vasculitis may lead to certain complications that include:

  • Low blood pressure
  • Hyperkalemia or high levels of potassium in blood
  • Tremors
  • Diabetes mellitus
  • Osteoporosis causing decreased bone density
  • Decreased muscle function
  • Long-term kidney dysfunction
  • Elevated risk for malignancy
  • Decrease in body immunity to fight infections

How is Rheumatoid Vasculitis Treated?

The treatment of Rheumatoid Vasculitis (RV) depends upon a variety of factors including:

  • Severity of the signs and symptoms
  • Regions of the body affected and the level of functioning of the affected organs
  • Overall health status of the individual and his/her age
  • Severity of the underlying rheumatoid arthritis (RA)

The main treatment of Rheumatoid Vasculitis is in treating the underlying rheumatoid arthritis. An effective treatment of RA can significantly reduce one’s risk for RV.

A multidisciplinary team of specialists that include general practitioners, rheumatologists, neurologists, pulmonologists, cardiovascular experts, nephrologists, dermatologists, among others, may be involved in the treatment, especially when systemic involvement is observed.

The treatment methods may include:

  • For mild RV involving only the skin and peripheral nerves, corticosteroid therapy using prednisone is considered. If necessary, medications may be used to regulate the immune system activity
  • In severe cases with far-reaching systemic symptoms involving many organs, high-dose steroids (such as cyclophosphamide) and immunosuppressive agents (such as methotrexate and azathioprine) may be used to control inflammation
  • Biologic agents using abatacept, rituximab, and monoclonal antibodies (anti-TNF antibodies), may be required for some severe cases
  • When individuals do not respond to the above-mentioned treatments, plasma exchange (plasmapheresis) or intravenous immunoglobulin (IVIG) therapy may be considered
  • Rarely, vascular surgery, such as vascular bypass procedures, vascular stent placements, and removal of blood clots (thrombectomy), are found to be beneficial
  • Physical therapy and occupational therapy

The treatment also depends on whether there are recurring episodes of the condition. The goal of the treatment is to control the inflammation in order to achieve remission (a state where no signs and symptoms are noted) and maintenance therapy to prevent episodes of relapses.

How can Rheumatoid Vasculitis be Prevented?

  • Currently, there are no methods available to prevent Rheumatoid Vasculitis. An early diagnosis and prompt treatment can help decrease the burden of the condition
  • Undertaking early and appropriate treatment of the underlying rheumatoid arthritis is very important; it can significantly lower one’s risk for Rheumatoid Vasculitis
  • Maintaining a healthy lifestyle with good eating habits, adequate physical activities, and avoidance of smoking or drinking is beneficial; an immediate stoppage of all forms of tobacco consumption is extremely important to prevent further aggravation of rheumatoid arthritis
  • Patients are required to be vigilant during the remission period and report any new symptoms promptly to the healthcare provider

Regular medical screening at periodic intervals with blood tests, scans, and physical examinations, are mandatory, due to the possibility of recurrence/relapse. Often several years of active vigilance is necessary.

What is the Prognosis of Rheumatoid Vasculitis? (Outcomes/Resolutions)

  • The prognosis of Rheumatoid Vasculitis (RV) depends upon a variety of factors including the:
    • Severity of the condition; degree of damage to the vital organs and body systems
    • Age and overall health of the individual
    • One’s response to treatment
    • Severity of the underlying rheumatoid arthritis (RA)
    • Whether it is an initial episode or a recurring episode
  • It is important to diagnose Rheumatoid Vasculitis early and institute appropriate treatment measures in order to control/improve the symptoms and prevent complication including organ injury and dysfunction
  • Individuals with extensive condition affecting several regions of the body have poorer prognosis, than individuals in whom the condition is confined to fewer body parts/regions. If left untreated, the condition has a potential to cause significant tissue injury and organ failure. There is also an increased risk for the development of cardiovascular diseases including brain stroke
  • Treating the underlying rheumatoid arthritis effectively is important to ensure a favorable prognosis. Usually, the medications used to treat rheumatoid arthritis help in appropriately managing Rheumatoid Vasculitis too. However, RA and RV are chronic conditions that need long-term treatment. Also, there may be relapses and recurrences that may adversely affect the overall prognosis and quality of one’s life

Even with treatment, Rheumatoid Vasculitis can relapse. If blood in cough or blood in urine is observed, then it is extremely important to contact the healthcare provider right away.

Additional and Relevant Useful Information for Rheumatoid Vasculitis:

The following article link will help you understand other Vein & Vascular Health:

https://www.dovemed.com/health-topics/vein-and-vascular-center/

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Krish Tangella MD, MBA

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