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

• UV (Urticarial Vasculitis)

What is Urticarial Vasculitis? (Definition/Background Information)

• Urticarial Vasculitis (UV) is a rare form of vasculitis that is mostly observed in adults. 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
• Urticarial Vasculitis primarily involves the small blood vessels of skin causing predominantly cutaneous symptoms resembling urticaria, which is characterized by red patches on skin, hives, itching sensation, and discoloration of skin. However, systemic involvement is seen in some individuals involving several organs, such as the lung, kidney, liver, and muscles and joints
• Urticarial Vasculitis are classified as two types, based on the level of certain complement proteins in blood. Complement proteins play an important role in the immune system of the body. The two types include:
  • Normocomplementemic Urticarial Vasculitis (NUV): It is the less severe form of UV and is characterized by normal complement protein levels in blood
  • Hypocomplementemic Urticarial Vasculitis (HUV): It is the more severe form of UV and is characterized by low complement protein levels in blood (below normal range)
• The exact cause of Urticarial Vasculitis is not well-understood, but it reportedly immune-mediated. Several autoimmune diseases, viral infections, use of certain medications, and malignancies are associated with the development of this form of vasculitis
• The blood levels of complement proteins correlated with the presenting signs and symptoms may help the healthcare provider diagnose Urticarial Vasculitis. A wide variety of tests and exams may be performed including blood and urine tests, radiological studies, and tissue biopsies, as needed, to understand the involvement of various body organs
• An early diagnosis and appropriate treatment of Urticarial Vasculitis can lead to a good prognosis. Generally, individuals with Hypocomplementemic Urticarial Vasculitis (skin and systemic involvement) have worse outcomes than those with Normocomplementemic Urticarial Vasculitis (skin with mild/no systemic involvement)

Who gets Urticarial Vasculitis? (Age and Sex Distribution)

• Urticarial Vasculitis is an uncommon condition that can affect both children and adults. However, most of the cases are reported in adults in the 30-40 year’ age range
  • Normocomplementemic subtype is seen with a slight female predominance (male-female ratio of 1:2)
  • Hypocomplementemic subtype is almost only observed in females
• It is observed worldwide and can affect people of all racial and ethnic backgrounds

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

The following risk factors (triggers or associated conditions) for Urticarial Vasculitis have been reported:

• Autoimmune disorders such as Sjögren's syndrome, systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), etc.
• Disorders involving immunoglobulins (proteins in blood) such as IgA and IgM monoclonal gammopathy
• Viral infections that include infectious mononucleosis (mono) and hepatitis B and C viral infections
• Use of certain medications including ACE inhibitors, antibiotics such as penicillin and sulfonamide, anti-depressants such as fluoxetine, and diuretics (thiazides)
• Malignancies such as leukemia, among other

In some cases, the risk factors are not clearly evident or present.

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 Urticarial Vasculitis? (Etiology)

• The exact cause of development of Urticarial Vasculitis is not known; researchers inform that it may be immune-mediated. It is reportedly a variant of cutaneous small-vessel vasculitis (CSVV)
• It may develop as a hypersensitivity reaction to certain stimuli, such as an inflammatory condition, infection, or medication use, triggering an abnormal immune reaction in the body

Urticarial Vasculitis is not transmitted from one individual to another; the condition is non-contagious.

What are the Signs and Symptoms of Urticarial Vasculitis?

The severity of signs and symptoms of Urticarial 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 depend on the type of Urticarial Vasculitis, the organs affected, and the presence of an underlying cause/trigger, if any.

The general signs and symptoms may include:

• Fever
• Skin rashes
• Headaches
• Bloodshot eyes
• Fatigue
• Loss of weight
• Weakness and malaise
• Pain that may affect the chest, back, or abdomen
• Shortness of breath

The signs and symptoms of Normocomplementemic Urticarial Vasculitis are less severe and mostly confined to the skin; rarely, are other organs involved (systemic disease). 

• The condition starts with bruises or skin eruptions resembling urticaria; urticaria causes weals (red skin sign with itching) and/or angioedema (swelling due to fluid accumulation)
• Itching and burning sensation is noted, including lymph node swelling
• Bleeding on scratching of skin
• Any skin of the body surface may be involved
• The red plaques may present white center; bleeding under skin (petechia) may be noted resulting in dark red discoloration
• The urticaria-like lesions usually last over a day and do not seem to migrate from one region to another
• In many individuals, these abnormal skin symptoms resolve on their own

Following the skin symptoms, systemic symptoms start that tend to mostly involve the lung and kidney. However, in Normocomplementemic Urticarial Vasculitis, involvement of organs other than skin is generally minimal to absent.

In Hypocomplementemic Urticarial Vasculitis, which is the more severe form of Urticarial Vasculitis, several vital organs of the body may be affected including the liver, gastrointestinal tract, joints, eyes, and the heart. It is important to note that the combination of signs and symptoms may vary from individual to individual.

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

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

• Nausea and vomiting
• 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 gastrointestinal (GI) system:

• Nausea and vomiting
• Fatigue and weakness
• Difficulty in swallowing
• Hiccups
• 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 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 eye (ocular system):

• Redness of the eyes
• Burning sensation in the eyes; eye irritation
• Foreign body sensation in the eye
• Excessive tearing (flowing of tears)
• Swollen eyelids
• Blurred vision
• Nyctalopia or night vision loss; unable to see in dim light
• Reduced visual acuity
• Temporary vision loss
• Eye pain
• Noticeable bleeding on the surface or inside the eye
• Eye floaters
• Flashes of light in vision of affected eye
• Light-sensitivity (photophobia)
• Impaired color vision
• Bulging of the eyes

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)

How is Urticarial Vasculitis Diagnosed?

A diagnosis of Urticarial Vasculitis will usually depend on the system or organ of the body that is affected and the underlying trigger, if any. The condition and its subtypes are typically diagnosed by measuring the levels of complement proteins (C1q complements) in blood. If their levels are normal, then it is Normocomplementemic Urticarial Vasculitis; but, if the levels are below normal or low, then it is Hypocomplementemic Urticarial Vasculitis.

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 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 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 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 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 eye (ocular system):

• Eye examination by an eye specialist (ophthalmologists or retinal experts)
• Blood tests and culture
• Fundoscopic (ophthalmoscopic) examination by an eye specialist, who examines the back part of the eye (or the fundus)
• Visual acuity test using a special and standardized test chart (Snellen chart)
• Slit-lamp examination: Examination of the eye structure using a special instrument called a slit-lamp. In this procedure, the pupils are dilated, and the internal eye structure is examined
• Tonometry: Measurement of intraocular pressure or eye fluid pressure, especially to detect conditions such as glaucoma
• Fundus fluorescein angiography (FFA): In this technique, the eye blood vessels are examined using a fluorescein dye
• Fundus autofluorescence (FAF): It is a non-invasive diagnostic technique to examine the fundus of the eye without using a fluorescent dye by a specialized fundus camera 
• Indocyanine green (ICG) angiography: It is used to examine the blood vessels of the choroid using a dye, called indocyanine green, particularly to study the choroid
• B-scan ultrasonography: Special ultrasound scan of the eye through a non-invasive diagnostic tool, to assess health of eye structures
• Electroretinogram (ERG): It is a technique to measure electrical activities in the retinal cells
• Optical coherence tomography (OCT) of eye: It is an ocular imaging technique to visualize the eye structure

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

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 Urticarial Vasculitis?

The complications of Urticarial Vasculitis that develop depend on the organs affected, severity of the signs and symptoms, and one’s response to treatment. Generally, Hypocomplementemic Urticarial Vasculitis presents more complications than Normocomplementemic Urticarial Vasculitis.

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

• Emotional stress due to cosmetic concerns
• 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 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)
• Chronic obstructive pulmonary disease (COPD)
• Irreversible lung damage
• Development of pneumothorax (collapsed lung)
• Respiratory failure
• Decreased quality of life

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 result in kidney damage and kidney failure

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

1. Inability to eat
2. Weight loss and malnutrition
3. GI tract bleeding
4. Perforation of the GI tract
5. Stricture formation of the food pipe

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 eye (ocular system) may include:

• Emotional stress due to cosmetic concerns
• Irreversible damage to the eye or eye structures
• Glaucoma
• Cataract formation
• Retinal detachment
• Complete loss of vision

In some minor cases, severe heart complications may be noted. Complications associated with the heart and blood vessels (cardiovascular system) may include:

• Severe arrhythmias
• Normal function of the heart is severely affected
• Congestive heart failure
• Increased risk for thromboembolism (blood clot obstructing a blood vessel)
• Chronic high blood pressure in the blood vessels of the lungs

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 Urticarial 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 Urticarial Vasculitis Treated?

The treatment of Urticarial Vasculitis depends upon a variety of factors including:

• Type of Urticarial Vasculitis - whether normocomplementemic or hypocomplementemic subtype
• 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 Urticarial Vasculitis

The main treatment of Urticarial Vasculitis includes treating the underlying cause. 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 for systemic conditions may include use of the following medications:
  • For infections, antiviral agents and antibiotics such as dapsone
  • Antimalarial medications such as hydroxychloroquine
  • Pain-relievers
  • Antihistamines and non-steroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen, naproxen, and indomethacin, for pain and swelling
  • Wound care for ulceration
  • Corticosteroid therapy, such as using oral prednisone, for severe and long-term Urticarial Vasculitis
• Immunosuppressive medications, such as methotrexate, azathioprine, cyclophosphamide, or ciclosporin, may be necessary if the condition is resistant to corticosteroids
• 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

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 Urticarial Vasculitis be Prevented?

Currently, there are no methods available to prevent Urticarial 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 Urticarial Vasculitis and is an important consideration
• Treating and managing the underlying condition that triggered the initial episode helps in preventing subsequent episodes
• 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 Urticarial Vasculitis? (Outcomes/Resolutions)

• The prognosis of Urticarial Vasculitis depends upon a variety of factors including the:
  • Subtype of Urticarial Vasculitis
  • Severity of the condition; 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
• Individuals with Normocomplementemic Urticarial Vasculitis have better outcomes than those with Hypocomplementemic Urticarial Vasculitis
  • The former subtype is usually mild and systemic involvement is minimal to absent; it is also known to be self-resolving, though some resolve slowly. The skin lesions are also known to get better and recede following appropriate treatment, sometimes after a few years
  • The latter subtype usually presents severe symptoms with the involvement of several organs and body systems. It is also generally difficult to treat. Thus, periodic follow-up and regular testing is important
• Nevertheless, Urticarial Vasculitis can be a chronic condition with relapses/recurrences and periods of remission. It is reported that 80% of the individuals respond well to corticosteroid therapy for the skin symptoms

Even with treatment, Urticarial 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 Urticarial Vasculitis:

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

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