IgA Vasculitis

IgA Vasculitis

Article
Bone, Muscle, & Joint
Vein & Vascular Health
+10
Contributed byKrish Tangella MD, MBAApr 14, 2020

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

  • Anaphylactoid Purpura
  • Henoch-Schönlein Purpura (HSP)
  • IgAV (IgA Vasculitis)

What is IgA Vasculitis? (Definition/Background Information)

  • IgA Vasculitis (IgAV) is a form of systemic vasculitis that is observed in young children. In this condition, inflammation of the small blood vessels is observed. It can cause damage to various body organs depending on the region affected, due to decreased blood and oxygen supply to the body region
  • IgA Vasculitis was earlier known as Henoch-Schönlein Purpura (HSP). It is characterized by three main manifestations known as the ‘classic triad’ that include red skin rashes (purpura), joint pain (arthritis), and abdominal pain, due to involvement of the skin, muscles and joints, and the gastrointestinal system
  • The exact cause of the condition is not understood, but it is reportedly immune-mediated. Immunoglobulin A (IgA), an antibody in blood that fights body infections, is known to play a significant role in the development of this condition, and hence, it is termed IgA Vasculitis
  • In a majority, IgA Vasculitis develops post bacterial or viral infection of the throat or stomach. A wide range of infections, such as caused by mycoplasma, adenovirus, or herpes simplex virus, are associated with IgA Vasculitis. In some, it is reported that certain medications may trigger the condition
  • In order to establish a diagnosis of IgA Vasculitis, a wide variety of tests and exams may be performed that include blood and urine tests, radiological studies such as CT and MRI scans, including a tissue biopsy, when needed
  • An early diagnosis and appropriate treatment of IgA Vasculitis can lead to a good prognosis. Most individuals recover in 6-8 weeks; the condition is usually self-limiting in most patients without treatment. Children have a much better overall prognosis than adults

Who gets IgA Vasculitis? (Age and Sex Distribution)

  • IgA Vasculitis is a common pediatric condition that affects young children. However, the condition can affect individuals of a wider age range including adults
  • About 90% of the cases are seen in children between the ages of 3 and 10 years, while 50% are observed in children below 6 years of age
  • Both males and females are affected with a slight male predominance (male-female ratio of 2:1)
  • It is observed worldwide and can affect people of all racial and ethnic backgrounds. In the US and UK, the incidence is around 14-20 cases per 100,000 population

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

A variety of risk factors are described in the medical literature for IgA Vasculitis. The primary risk factors are reportedly infections.

  • Viral infections such as:
    • Hepatitis B virus infection
    • Measles
    • Mumps
    • Rubella
  • Infections caused by viruses such as:
    • Adenovirus
    • Coxsackievirus
    • Herpes simplex virus
    • Parvovirus b19
  • Infections caused by bacteria such as:
    • Helicobacter pylori
    • Mycoplasma
    • Staphylococcus aureus
    • Streptococci

The other risk factors for IgA Vasculitis include:

  • Use of certain medications such as:
    • ACE inhibitors
    • Antibiotics
    • Anti-inflammatory medications
  • Vaccinations
  • Food allergies
  • Presence of certain malignancies
  • Insect bites
  • Colder temperatures relatively show an increased incidence of the condition; thus, a greater number of cases are observed during autumn and winter

In some cases, the risk factors for IgA Vasculitis are not clearly recognized.

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 IgA Vasculitis? (Etiology)

The exact cause of development of IgA Vasculitis is not known. Researchers inform that it may be a type of autoimmune disorder. 

  • In this condition, there are immune deposits consisting of IgA protein molecules. The role of immunoglobulin A (IgA) antibody that is found in blood, is to fight infections. IgA Vasculitis may develop as a hypersensitivity reaction to certain stimuli (such as an infection or medication use) triggering an abnormal immune reaction in the body
  • According to medical literature, IgA Vasculitis is known to develop following infection of the gastrointestinal or respiratory system (usually a throat infection) in 3 out of 4 cases. A variety of viral and bacterial infections have been implicated

The presence of skin lesions that ulcerate and bleed in IgA Vasculitis, does not indicate that the condition is contagious. It is not transmitted from one individual to another.

What are the Signs and Symptoms of IgA Vasculitis?

The severity of signs and symptoms of IgA Vasculitis may vary from one individual/child 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 of IgA Vasculitis depend on the organs affected and the presence of an underlying cause/trigger, if any.

The classic triad of IgA Vasculitis noted in many are:

  • Red rashes - these are always observed in children and those affected
  • Joint pain (arthritis) is observed in 4 out of 5 cases
  • Abdominal pain is observed in nearly two-thirds of the cases

In many, fever, headache, and muscle pain are noted for a prolonged period before onset of other main symptoms of the condition.

The vasculitis associated general signs and symptoms may include

  • Fatigue
  • Loss of weight
  • Weakness
  • Pain that may affect the chest or back
  • Shortness of breath

In general, IgA Vasculitis is a systemic disorder involving different organs of the body. Usually the muscles and joints, gastrointestinal tract, kidney, skin and mucus membranes are affected. Involvement of the central nervous system (CNS) and lungs are noted in some cases. It is important to note that the combination of signs and symptoms may vary from individual to individual.

Signs and symptoms related to the skin and underlying tissue (integumentary system):

  • Red rashes (purpura), mostly on the lower part of the body, feet, legs, and genitals and buttocks; these are sometimes seen on the upper body such as the arms, elbows, chest and back, including the face
  • Elevated swellings
  • Pain and discomfort
  • Itchiness of skin causing irritability
  • Dryness of skin
  • Discoloration of skin (pigmentation changes)
  • Bleeding on scratching of skin
  • Any skin of the body surface may be involved

Signs and symptoms related to the muscles and joints (musculoskeletal system):

  • Inflammation of the muscles and joints
  • Muscle and joint pain, involving one or more joints; mostly the knees and ankles are affected, and sometimes the elbows
  • Tenderness of the muscles
  • Muscle stiffness and spasms
  • Swollen joints
  • Joint stiffness
  • Muscle weakness, especially involving the upper or lower limbs
  • Loss of muscle mass
  • Muscle contracture
  • Difficulty climbing stairs and walking
  • Difficulty with other motor skills, including jumping, hopping, and running
  • Difficulty in bending or stretching one’s back
  • Limited range of motion; decreased joint function
  • The condition may involve any joint in the body such as the knee, elbow, shoulder, hip, etc.

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)
  • Loss of appetite
  • Stomach or abdominal pain and discomfort
  • Abdominal bloating and distension
  • Irregular bowel movements
  • Passing gas; belching or flatus
  • Constipation or diarrhea
  • Blood or mucus in stool; this may precede the rashes
  • 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
  • Pain while urination, frequent urge to urinate
  • Presence of blood in the urine
  • 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 due to kidney problems

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
  • Numbness and tingling sensation
  • Muscle weakness; 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
  • Dizziness; vertigo
  • Confusion
  • Fainting
  • Change in behavior
  • Mental impairment
  • Memory loss

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
  • Wheezing, exhibiting a high-pitched sound while breathing (stridor)
  • Changes to one’s voice
  • Hoarseness
  • Loss of voice

How is IgA Vasculitis Diagnosed?

A diagnosis of IgA Vasculitis will usually depend on the system or organ of the body that is affected and the underlying trigger, if any. The diagnosis 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 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 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 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 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) and peripheral nervous system (cerebrovascular 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

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

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

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 IgA Vasculitis?

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

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

  • Emotional stress due to cosmetic concerns
  • Discomfort, irritability of the affected skin
  • Bleeding and ulceration
  • Severe pain and itchiness of skin leading to acute discomfort and irritation, including sleep disruption
  • The lesions may occasionally develop bacterial or fungal infections
  • Permanent scarring

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

  • Chronic pain and weakness of the joint affecting the quality of life; however, joint pain heals well with treatment and does not cause any permanent injury
  • Formation of scar tissue in the muscle and joint tissue
  • Immobilization due to loss of movement
  • Permanent disability

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

  1. Inability to eat
  2. Weight loss and malnutrition
  3. GI tract bleeding may be seen in 1 in 3 cases
  4. Perforation of the GI tract
  5. Stricture formation of the food pipe
  6. Intussusception - where the intestine folds into itself causing obstruction and injury

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); inflammation of the kidneys can cause small amounts of blood/protein in urine (in 2 out of 5 cases)
  • Erectile dysfunction
  • Urinary incontinence (involuntary dripping or leakage of urine)
  • Severe cases may result in kidney damage and kidney failure, especially in adults

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

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

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 IgA 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 IgA Vasculitis Treated?

The treatment of IgA Vasculitis 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
  • Underlying condition triggering IgA Vasculitis

The main treatment of IgA Vasculitis involves addressing the underlying cause. In individuals who have a known trigger, treatment of the underlying causative condition usually brings about a resolution. Older individuals with severe symptoms require prolonged treatment. The condition is frequently recurrent in such individuals too.

If a clearly identifiable cause is not known, then the primary role of treatment is to reduce inflammation. Often, a multidisciplinary team of specialists that include general practitioners, rheumatologists, neurologists, pulmonologists, cardiovascular experts, nephrologists, dermatologists, ophthalmologists, among others, may be involved in the treatment.

The treatment methods for the condition may include:

  • Symptomatic treatment may include antibiotics and antiviral agents for infection, use of pain-relievers such as acetaminophen/paracetamol, anti-inflammatory drugs such as ibuprofen for pain and swelling, wound care for severe ulceration, etc.
  • Corticosteroid therapy using prednisone; the use of steroids is avoided in children
  • Immunosuppressive medications may be necessary for severe inflammation, particularly if the kidneys are affected
  • Biologic agents using specific monoclonal antibodies to reduce inflammation
  • In cases with severe symptoms, or when the affected individuals do not respond to the above-mentioned treatments, plasma exchange (plasmapheresis) or intravenous immunoglobulin (IVIG) therapy may be considered
  • Kidney dialysis and kidney transplant may be needed in some rare cases, mostly in adult patients

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 IgA Vasculitis be Prevented?

Currently, there are no methods available to prevent IgA Vasculitis. An early diagnosis and prompt treatment can help decrease the burden of the condition.

  • Undertaking early and appropriate treatment of the underlying infection or condition (potential risk factors) may help in lowering one’s risk for IgA Vasculitis and is an important consideration
  • Treating and managing the underlying condition that triggered the initial episode helps in preventing subsequent episodes
  • If the condition is triggered by medication (drug-induced IgA Vasculitis), stopping of the medication and refraining from further using the medication can help in preventing its recurrence
  • Maintaining a healthy lifestyle with good eating habits, adequate physical activities, and avoidance of smoking or drinking is beneficial
  • Ensuring that individuals, especially children, are up-to-date on their vaccinations is important
  • 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 IgA Vasculitis? (Outcomes/Resolutions)

  • The prognosis of IgA Vasculitis depends upon a variety of factors including the:
    • Severity of IgA Vasculitis; degree of damage to the vital organs and body systems
    • Age and overall health of the individual
    • Underlying triggering factor
    • One’s response to treatment: Individuals who respond to treatment usually have a better prognosis than those who do not respond well to treatment
    • Whether it is an initial episode or a recurring episode
  • In a majority of individuals, especially children below 16 years of age, IgA Vasculitis is self-resolving in 30-60 days even without treatment. The prognosis is generally excellent and the overall recovery rates are between 90-95%. Moreover, many patients are known to respond well to appropriate treatment
  • Nearly 35% of the cases are known to relapse after a few months (usually 4 months) of first incidence of attack. The severity of symptoms usually decreases on subsequent attacks. Thus, periodic follow-up and regular testing is important
  • In adults, it may be chronic condition and may last over 2 years; IgA Vasculitis tends to relapse and remit presenting more complications. In 1% of the cases, chronic kidney disease may develop; those with severe kidney damage may need prolonged treatment and checkups

Even with treatment, IgA 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 IgA 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

Pathology, Medical Editorial Board, DoveMed Team

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