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Aortitis

Updated : December 27, 2023





Background

  • Aortitis is a rare but serious medical condition characterized by inflammation of the aorta, the aortic walls may become weaker due to this inflammation, leading to potential complications such as aneurysm, dissection, or other cardiovascular issues. 
  • The condition can manifest in different forms, including infectious and non-infectious types, each with its own underlying causes and contributing factors. While aortitis is relatively uncommon, its impact on cardiovascular health can be severe if left untreated. 

Epidemiology

Incidence: Estimates of 1 to 3 new cases per million people per year in the US vary greatly, according to data that is highly variable.   

Age and Sex: 

Vasculitides: Most common in young adults. 

Infectious: It can occur at any age, depending on the specific infection. 

Geographic Variations: Prevalence appears to differ geographically, with potentially higher rates in Asia for certain types like Takayasu arteritis. 

 

Anatomy

Pathophysiology

  • Inflammatory Response: In response to the triggering factor, immune cells, including T-lymphocytes and macrophages, infiltrate the aortic wall. The inflammatory process is started and sustained by these cells by the release of cytokines and other inflammatory mediators. 
  • Damage to Aortic Wall: Continuous inflammation leads to damage of the intima, media, and adventitia of the aorta. This damage weakens the vessel’s structural integrity. 
  • Fibrosis and Scarring: Chronic inflammation stimulates fibroblasts to produce collagen and other connective tissue components, leading to fibrosis and scarring within the aortic wall. This can cause thickening and stiffness of the vessel. 
  • Vessel Wall Weakness and Complications: As the inflammatory process persists, the aortic wall may weaken, leading to complications such as aneurysm formation, dissection, or rupture. These complications can be life-threatening. 

Etiology

  • Vasculitides: These autoimmune disorders primarily target blood vessels, including the aorta. The most common vasculitides associated with aortitis are: 
  • Giant cell arteritis: Primarily affects adults over 50 years old and typically involves the ascending aorta. 
  • Takayasu arteritis: Mostly affects young women and adolescents, often targeting the arch and descending aorta. 
  • Microscopic polyangiitis: Can lead to widespread aortitis involving multiple aortic segments. 
  • Other ANCA-associated vasculitides: Granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis can sometimes involve the aorta. 
  • Infections: While less common than vasculitides, infectious agents can directly invade the aortic wall, triggering inflammation.  
  • Bacteria: Mycobacterium tuberculosis, Salmonella, Staphylococcus aureus, Streptococcus pneumoniae 
  • Fungi: Aspergillus fumigatus, Candida albicans 
  • Viruses: Cytomegalovirus, Epstein-Barr virus 

Genetics

Prognostic Factors

  • Disease Severity: The extent and severity of aortitis can affect prognosis. The involvement of the aortic arch, ascending aorta, or involvement of major branches can pose more significant risks and complications. 
  • Complications: The development of complications such as aneurysm formation, aortic dissection, or rupture can significantly worsen the prognosis. These complications may require immediate medical or surgical intervention. 
  • Coexisting Health Conditions: The presence of other health conditions or comorbidities, such as hypertension, diabetes, or cardiovascular diseases, can affect the overall prognosis and management of aortitis. 

Clinical History

  • Age group 

Aortitis, or inflammation of the aorta, can occur at any age, but it is more commonly observed in adults rather than in children. The condition can affect individuals across a wide range of ages, predominantly in adults between the ages of 30 and 60. However, it’s important to note that aortitis can also occur in individuals outside this age range, including younger adults and older individuals. 

Physical Examination

Cardiovascular: 

  • Blood pressure: 
  • Low blood pressure: It can occur in severe cases due to compromised blood flow from aortic narrowing or regurgitation. 

Pulse: 

  • Weak or diminished pulses: In cases where aortitis affects specific arterial branches, pulses in the corresponding extremities may be weaker or absent. 
  • Heart murmurs: Various murmurs can be present depending on the location and severity of aortitis, including aortic stenosis murmur, aortic regurgitation murmur, and aortic insufficiency murmur. 
  • Other findings: 
  • Claudication: Leg pain on exertion due to decreased blood flow to the legs can occur in severe cases of aortitis affecting abdominal aorta or its branches. 
  • Neurological findings: If aortitis involves cerebral arteries, neurological symptoms like stroke or transient ischemic attacks might be present. 

Age group

Associated comorbidity

  • Autoimmune Diseases: Ankylosing spondylitis, rheumatoid arthritis, giant cell arteritis, takayasu arteritis, and systemic lupus erythematosus are among the autoimmune diseases that have been linked to aortitis. In these cases, inflammation results from the immune system of the body inadvertently targeting the arteries. 
  • Infections: Certain infections, such as syphilis, tuberculosis, bacterial endocarditis, and other bacterial or fungal infections, can cause aortitis by directly infecting the aortic wall. 
  • Vasculitis: Aortitis is commonly linked to vasculitis conditions characterized by inflammation of blood vessels. Diseases like takayasu arteritis and giant cell arteritis specifically affect large vessels, including the aorta. 
  • Atherosclerosis: Chronic inflammation associated with atherosclerosis, a condition where plaque buildup occurs in arteries, can lead to aortitis. Atherosclerosis can cause damage to the aortic wall, triggering an inflammatory response. 
  • Trauma: Injuries or trauma to the aorta, such as blunt chest trauma from accidents or surgeries involving the aorta, can lead to inflammation. 

Associated activity

Acuity of presentation

  • Symptoms onset: The onset of symptoms can range from acute to chronic depending on the underlying cause. In acute cases, symptoms may develop rapidly and be more severe, while in chronic cases, symptoms might appear gradually and progress slowly over time. 
  • Severity of symptoms: Acute aortitis may present with more intense symptoms such as sudden chest pain, high fever, malaise, and signs of systemic inflammation. Chronic aortitis may have more subtle symptoms initially, such as mild chest discomfort, fatigue, or vague constitutional symptoms. 
  • Specific symptoms: Common symptoms of aortitis can include chest pain, fever, fatigue, weight loss, night sweats, and sometimes symptoms related to the involvement of other organs if the condition is part of a systemic disease. 
  • Systemic manifestations: Depending on the underlying cause like infections like syphilis, tuberculosis, or autoimmune diseases like giant cell arteritis, aortitis can have associated systemic manifestations such as joint pain, skin rashes, eye problems, or neurological symptoms. 

Differential Diagnoses

  • Giant Cell Arteritis: GCA is a type of vasculitis affecting medium and large arteries, including the aorta. It commonly involves the branches of the carotid artery and can cause aortitis. 
  • Infectious Aortitis: Infections, such as bacterial as syphilis, tuberculosis, endocarditis, fungal, or viral like herpes, HIV, can cause aortitis. These infections can directly affect the aortic wall, leading to inflammation. 
  • Behcet’s Disease: This is a multisystem inflammatory disorder that can cause vasculitis affecting arteries and veins. Aortitis can be a manifestation of Behcet’s disease. 
  • Takayasu’s Arteritis: The aorta and its main branches are the main organs affected by this uncommon form of vasculitis. It can lead to artery constriction, scarring and inflammation. 

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

  • Identification of Underlying Cause: Accurate diagnosis is crucial for determining the appropriate treatment. This may involve a comprehensive medical history, physical examination, imaging studies, blood tests, and sometimes a biopsy of the affected tissue. 
  • Medical Management: 
  • Anti-Inflammatory Medications: Non-steroidal anti-inflammatory drugs or corticosteroids could be recommended to control discomfort and reduces inflammation. 
  • Immunosuppressive therapy: In cases where aortitis is associated with autoimmune diseases like giant cell arteritis or takayasu arteritis, more potent immunosuppressive drugs such as methotrexate, azathioprine, or biologics might be used to control the immune response. 
  • Antibiotics or Antivirals: If an infectious agent causes aortitis, targeted antimicrobial therapy will be necessary. 
  • Management of Complications: 
  • Aneurysm or Dissection: Aortitis can lead to weakening of the aortic wall, potentially causing aneurysms or dissections. Surgical intervention, such as open repair or endovascular procedures, might be required to address these complications and prevent life-threatening events. 
  • Monitoring: Regular imaging studies are often recommended to monitor the progression of the disease and to detect any complications early. 
  • Lifestyle Modifications: Patients may be advised to adopt certain lifestyle changes to manage symptoms and improve overall health.  
  • Follow-up Care: Long-term management often requires ongoing monitoring and adjustments to treatment plans. Regular follow-up appointments with healthcare providers are crucial to assess the response to treatment, manage side effects of medications, and address any new symptoms or complications. 
  • Multidisciplinary Approach: Aortitis management often involves a team of healthcare professionals, including rheumatologists, cardiologists, vascular surgeons, infectious disease specialists, and others, depending on the specific needs of the patient. 

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

Lifestyle modifications in treating aortitis

  • Dietary Modifications: A heart-healthy diet, low in saturated fats, cholesterol, and sodium, is beneficial for individuals with aortitis.  
  • Regular Exercise: Engaging in regular physical activity can help improve cardiovascular health, strengthen the heart, and reduce inflammation.  
  • Monitoring and Managing Risk Factors: Managing underlying conditions such as high blood pressure, high cholesterol, diabetes, and obesity can help reduce the risk of aortitis and its complications. Regular monitoring and appropriate management of these risk factors are essential. 
  • Regular Follow-ups and Medical Care: It’s important for individuals diagnosed with aortitis to have regular follow-up appointments with their healthcare providers. This helps in monitoring the condition, adjusting treatment as necessary, and preventing potential complications. 
  • Avoiding Triggers: If aortitis is related to an autoimmune condition, avoiding triggers that may exacerbate the inflammation or autoimmune response is important. This may involve identifying and managing stress, infections, or other factors that can worsen the condition. 
  • Education and Support: Education about the condition, its causes, symptoms, and management are crucial for individuals with aortitis. Support groups or counselling may also be beneficial in coping with the challenges associated with the condition. 

Effectiveness of corticosteroids in treating aortitis

  • prednisone (Deltasone, Sterapred, Orasone) 
  • It might reduce inflammation by inhibiting activity of leukocytes and decreasing capillary permeability. 
  • Doses should be taken every day by those actively manifesting takayasu arteritis, with this therapy, 75% of patients recover and those individuals who did not respond or relapse after tapering required to take other immunosuppressants. 

Use of Immunosuppressants in treating aortitis

  • mycophenolate (CellCept) 
  • Mycophenolate is an immunosuppressive medication that is commonly used in the management of various autoimmune and inflammatory conditions.  
  • It prevents the synthesis of purines and the growth of lymphocytes, because of its decreased toxicity, this regimen is a compelling substitute. 
  • cyclophosphamide (Cytoxan) 
  • Cyclophosphamide is a potent immunosuppressive medication that belongs to the class of alkylating agents. 
  • DNA cross-linking may be the mechanism of action of active metabolites, and this can impede the growth of both neoplastic cells & normal cells. 

Effectiveness of anti-tumor necrosis factor agents in treating aortitis

  • etanercept (Enbrel) 
  • It is classified as tumor necrosis factor-alpha (TNF-alpha) inhibitors. They work by targeting and inhibiting the action of TNF-alpha, a pro-inflammatory cytokine involved in the immune response. 
  • infliximab (Remicade) 
  • It is classified as tumor necrosis factor-alpha (TNF-alpha) inhibitors. They work by targeting and inhibiting the action of TNF-alpha, a pro-inflammatory cytokine involved in the immune response. 
  • It is used in conjunction with methotrexate for patients whose response to methotrexate monotherapy was insufficient. 
  • 5- Use of antibiotics in treating aortitis: Specialty:  
  • Cardiology 
  • minocycline (Minocin, Dynacin) 
  • Antibiotics like minocycline can be effective against certain bacterial infections, they might not address non-infectious causes of aortitis, such as those related to autoimmune conditions or other underlying diseases.  
  •  

Aortic Repair or Replacement

  • Open Surgery: In a traditional open surgical repair, the damaged portion of the aorta is accessed by making an incision in the chest or abdomen. The damaged section may be repaired using various techniques, including removing the diseased portion and suturing the healthy ends together, or replacing the damaged segment with a synthetic graft. 
  • Endovascular Repair: During endovascular procedures, a tiny incision in the groin is used to introduce a catheter, which is then guided through blood arteries to the location of the damaged aorta. Using specialized instruments, a stent graft is placed within the aorta to reinforce the weakened or damaged area and restore blood flow. This technique is less invasive than open surgery and may be suitable for certain types of aortic disease. 

Role of management in treating aortitis

  • Diagnosis and Evaluation: 
  • Clinical Assessment: Based on symptoms like chest or back pain, fever, weight loss, and other systemic symptoms. 
  • Imaging Tests: CT scans, MRI, ultrasound, or angiography to visualize the aorta and detect inflammation. 
  • Treatment: 
  • Medication: Depending on the cause and severity, treatment may involve: 
  • Corticosteroids or immunosuppressive drugs to reduce inflammation in conditions like Takayasu arteritis. 
  • Antibiotics for infectious causes. 
  • Disease-modifying antirheumatic drugs or biologic therapies. 
  • Pain Management: Medications to alleviate pain and discomfort. 
  • Blood Pressure Control: Keeping blood pressure within a healthy range to reduce stress on the aorta. 
  • Monitoring and Follow-up: 
  • Regular Medical Check-ups: Monitoring symptoms and disease progression. 
  • Imaging Follow-ups: Periodic imaging tests to evaluate the aorta for any changes or progression of inflammation. 
  • Laboratory Tests: Monitoring inflammatory markers and overall health. 
  • Lifestyle Modifications: 
  • Healthy Diet: Emphasizing a diet low in sodium and saturated fats to manage blood pressure and overall heart health. 
  • Regular Exercise: Under guidance and moderation, to maintain cardiovascular fitness. 
  • Smoking Cessation: Encouraging quitting smoking as it exacerbates inflammation and increases the risk of complications. 
  • Surgical or Interventional Options: 
  • Aortic Repair: In severe cases with aneurysms, tears, or significant narrowing, surgical interventions like aortic repair, bypass surgery or stent placement might be required. 
  • Endovascular Procedures: Minimally invasive procedures like endovascular stent grafting or angioplasty can sometimes be options for certain types of aortic issues. 
  • Education and Support: 
  • Patient Education: Providing information about the condition, treatment, and lifestyle modifications. 
  • Support Groups: Connecting patients with support groups or resources for emotional support and shared experiences. 
  • Long-term Management: 
  • Continued Care: Lifelong management might be necessary, especially in chronic conditions like certain forms of vasculitis. 
  • Adherence to Treatment: Encouraging compliance with medications and follow-up appointments. 

Medication

Media Gallary

Aortitis

Updated : December 27, 2023




  • Aortitis is a rare but serious medical condition characterized by inflammation of the aorta, the aortic walls may become weaker due to this inflammation, leading to potential complications such as aneurysm, dissection, or other cardiovascular issues. 
  • The condition can manifest in different forms, including infectious and non-infectious types, each with its own underlying causes and contributing factors. While aortitis is relatively uncommon, its impact on cardiovascular health can be severe if left untreated. 

Incidence: Estimates of 1 to 3 new cases per million people per year in the US vary greatly, according to data that is highly variable.   

Age and Sex: 

Vasculitides: Most common in young adults. 

Infectious: It can occur at any age, depending on the specific infection. 

Geographic Variations: Prevalence appears to differ geographically, with potentially higher rates in Asia for certain types like Takayasu arteritis. 

 

  • Inflammatory Response: In response to the triggering factor, immune cells, including T-lymphocytes and macrophages, infiltrate the aortic wall. The inflammatory process is started and sustained by these cells by the release of cytokines and other inflammatory mediators. 
  • Damage to Aortic Wall: Continuous inflammation leads to damage of the intima, media, and adventitia of the aorta. This damage weakens the vessel’s structural integrity. 
  • Fibrosis and Scarring: Chronic inflammation stimulates fibroblasts to produce collagen and other connective tissue components, leading to fibrosis and scarring within the aortic wall. This can cause thickening and stiffness of the vessel. 
  • Vessel Wall Weakness and Complications: As the inflammatory process persists, the aortic wall may weaken, leading to complications such as aneurysm formation, dissection, or rupture. These complications can be life-threatening. 
  • Vasculitides: These autoimmune disorders primarily target blood vessels, including the aorta. The most common vasculitides associated with aortitis are: 
  • Giant cell arteritis: Primarily affects adults over 50 years old and typically involves the ascending aorta. 
  • Takayasu arteritis: Mostly affects young women and adolescents, often targeting the arch and descending aorta. 
  • Microscopic polyangiitis: Can lead to widespread aortitis involving multiple aortic segments. 
  • Other ANCA-associated vasculitides: Granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis can sometimes involve the aorta. 
  • Infections: While less common than vasculitides, infectious agents can directly invade the aortic wall, triggering inflammation.  
  • Bacteria: Mycobacterium tuberculosis, Salmonella, Staphylococcus aureus, Streptococcus pneumoniae 
  • Fungi: Aspergillus fumigatus, Candida albicans 
  • Viruses: Cytomegalovirus, Epstein-Barr virus 
  • Disease Severity: The extent and severity of aortitis can affect prognosis. The involvement of the aortic arch, ascending aorta, or involvement of major branches can pose more significant risks and complications. 
  • Complications: The development of complications such as aneurysm formation, aortic dissection, or rupture can significantly worsen the prognosis. These complications may require immediate medical or surgical intervention. 
  • Coexisting Health Conditions: The presence of other health conditions or comorbidities, such as hypertension, diabetes, or cardiovascular diseases, can affect the overall prognosis and management of aortitis. 
  • Age group 

Aortitis, or inflammation of the aorta, can occur at any age, but it is more commonly observed in adults rather than in children. The condition can affect individuals across a wide range of ages, predominantly in adults between the ages of 30 and 60. However, it’s important to note that aortitis can also occur in individuals outside this age range, including younger adults and older individuals. 

Cardiovascular: 

  • Blood pressure: 
  • Low blood pressure: It can occur in severe cases due to compromised blood flow from aortic narrowing or regurgitation. 

Pulse: 

  • Weak or diminished pulses: In cases where aortitis affects specific arterial branches, pulses in the corresponding extremities may be weaker or absent. 
  • Heart murmurs: Various murmurs can be present depending on the location and severity of aortitis, including aortic stenosis murmur, aortic regurgitation murmur, and aortic insufficiency murmur. 
  • Other findings: 
  • Claudication: Leg pain on exertion due to decreased blood flow to the legs can occur in severe cases of aortitis affecting abdominal aorta or its branches. 
  • Neurological findings: If aortitis involves cerebral arteries, neurological symptoms like stroke or transient ischemic attacks might be present. 
  • Autoimmune Diseases: Ankylosing spondylitis, rheumatoid arthritis, giant cell arteritis, takayasu arteritis, and systemic lupus erythematosus are among the autoimmune diseases that have been linked to aortitis. In these cases, inflammation results from the immune system of the body inadvertently targeting the arteries. 
  • Infections: Certain infections, such as syphilis, tuberculosis, bacterial endocarditis, and other bacterial or fungal infections, can cause aortitis by directly infecting the aortic wall. 
  • Vasculitis: Aortitis is commonly linked to vasculitis conditions characterized by inflammation of blood vessels. Diseases like takayasu arteritis and giant cell arteritis specifically affect large vessels, including the aorta. 
  • Atherosclerosis: Chronic inflammation associated with atherosclerosis, a condition where plaque buildup occurs in arteries, can lead to aortitis. Atherosclerosis can cause damage to the aortic wall, triggering an inflammatory response. 
  • Trauma: Injuries or trauma to the aorta, such as blunt chest trauma from accidents or surgeries involving the aorta, can lead to inflammation. 
  • Symptoms onset: The onset of symptoms can range from acute to chronic depending on the underlying cause. In acute cases, symptoms may develop rapidly and be more severe, while in chronic cases, symptoms might appear gradually and progress slowly over time. 
  • Severity of symptoms: Acute aortitis may present with more intense symptoms such as sudden chest pain, high fever, malaise, and signs of systemic inflammation. Chronic aortitis may have more subtle symptoms initially, such as mild chest discomfort, fatigue, or vague constitutional symptoms. 
  • Specific symptoms: Common symptoms of aortitis can include chest pain, fever, fatigue, weight loss, night sweats, and sometimes symptoms related to the involvement of other organs if the condition is part of a systemic disease. 
  • Systemic manifestations: Depending on the underlying cause like infections like syphilis, tuberculosis, or autoimmune diseases like giant cell arteritis, aortitis can have associated systemic manifestations such as joint pain, skin rashes, eye problems, or neurological symptoms. 
  • Giant Cell Arteritis: GCA is a type of vasculitis affecting medium and large arteries, including the aorta. It commonly involves the branches of the carotid artery and can cause aortitis. 
  • Infectious Aortitis: Infections, such as bacterial as syphilis, tuberculosis, endocarditis, fungal, or viral like herpes, HIV, can cause aortitis. These infections can directly affect the aortic wall, leading to inflammation. 
  • Behcet’s Disease: This is a multisystem inflammatory disorder that can cause vasculitis affecting arteries and veins. Aortitis can be a manifestation of Behcet’s disease. 
  • Takayasu’s Arteritis: The aorta and its main branches are the main organs affected by this uncommon form of vasculitis. It can lead to artery constriction, scarring and inflammation. 
  • Identification of Underlying Cause: Accurate diagnosis is crucial for determining the appropriate treatment. This may involve a comprehensive medical history, physical examination, imaging studies, blood tests, and sometimes a biopsy of the affected tissue. 
  • Medical Management: 
  • Anti-Inflammatory Medications: Non-steroidal anti-inflammatory drugs or corticosteroids could be recommended to control discomfort and reduces inflammation. 
  • Immunosuppressive therapy: In cases where aortitis is associated with autoimmune diseases like giant cell arteritis or takayasu arteritis, more potent immunosuppressive drugs such as methotrexate, azathioprine, or biologics might be used to control the immune response. 
  • Antibiotics or Antivirals: If an infectious agent causes aortitis, targeted antimicrobial therapy will be necessary. 
  • Management of Complications: 
  • Aneurysm or Dissection: Aortitis can lead to weakening of the aortic wall, potentially causing aneurysms or dissections. Surgical intervention, such as open repair or endovascular procedures, might be required to address these complications and prevent life-threatening events. 
  • Monitoring: Regular imaging studies are often recommended to monitor the progression of the disease and to detect any complications early. 
  • Lifestyle Modifications: Patients may be advised to adopt certain lifestyle changes to manage symptoms and improve overall health.  
  • Follow-up Care: Long-term management often requires ongoing monitoring and adjustments to treatment plans. Regular follow-up appointments with healthcare providers are crucial to assess the response to treatment, manage side effects of medications, and address any new symptoms or complications. 
  • Multidisciplinary Approach: Aortitis management often involves a team of healthcare professionals, including rheumatologists, cardiologists, vascular surgeons, infectious disease specialists, and others, depending on the specific needs of the patient.