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Vasculitis

Vasculitis refers to a group of rare disorders characterized by inflammation of blood vessels, including arteries, veins, and capillaries. This inflammation can lead to thickening, narrowing, weakening, or scarring of the vessel walls, which disrupts normal blood flow and can result in tissue and organ damage. Vasculitis can affect a single organ or multiple systems and varies significantly in severity, ranging from mild to life-threatening conditions.

There are many types of vasculitis, classified based on the size of the blood vessels affected (large, medium, or small). While the exact cause is often unknown, vasculitis is commonly linked to autoimmune activity, where the immune system mistakenly attacks healthy blood vessels.

Types of Vasculitis

Large vessel vasculitis:

  • Giant Cell Arteritis (Temporal Arteritis)*: Usually affects arteries in the head, especially the temples.
  • Takayasu Arteritis: Primarily affects the aorta and its major branches.

Medium vessel vasculitis:

  • Polyarteritis Nodosa: Affects medium-sized arteries, often involving kidneys, skin, nerves, and gastrointestinal tract.
  • Kawasaki Disease: Primarily affects children and may cause coronary artery inflammation.

Small vessel vasculitis:

  • Granulomatosis with Polyangiitis (Wegener’s): Affects respiratory tract and kidneys.
  • Microscopic Polyangiitis: Involves lungs, kidneys, and nerves.
  • Henoch-Schönlein Purpura (IgA Vasculitis): Common in children, with rash, joint pain, and kidney involvement.

The exact cause of vasculitis is often unknown, but several triggers and risk factors have been identified:

  • Autoimmune disorders: Lupus, rheumatoid arthritis, and scleroderma are associated with vasculitis.
  • Infections: Hepatitis B and C, HIV, and other chronic infections can trigger vasculitis.
  • Medications: Certain antibiotics and drugs can induce hypersensitivity vasculitis.
  • Genetic factors: Family history may increase susceptibility.
  • Environmental exposures: Toxins, allergens, or even vaccines in rare cases.

Pathophysiology

Vasculitis involves immune-mediated injury to blood vessels. The body’s immune system mistakenly identifies components of blood vessels as foreign and mounts an inflammatory response. This inflammation can compromise the structural integrity of the vessel wall, leading to:

  • Vascular occlusion due to thrombosis or scarring
  • Hemorrhage from weakened vessel walls
  • Ischemia and infarction of affected tissues and organs

The exact immune mechanisms vary with each type of vasculitis but often involve autoantibodies, immune complex deposition, or direct T-cell-mediated cytotoxicity.

Symptoms

Symptoms of vasculitis can vary widely depending on the type and organs involved, but general symptoms include:

  • Fever
  • Fatigue
  • Weight loss
  • Muscle and joint pain
  • Night sweats
  • Skin rashes or purpura

Organ-specific symptoms may include:

  • Nervous system: Numbness, weakness, or stroke-like symptoms
  • Kidneys: Blood in urine, high blood pressure
  • Lungs: Shortness of breath, coughing up blood
  • GI tract: Abdominal pain, bleeding
  • Eyes: Vision changes or redness

Diagnosis

Diagnosing vasculitis is complex and often requires a multidisciplinary approach. The diagnosis is based on clinical evaluation, laboratory testing, and imaging.

Blood tests:

  • Elevated ESR and CRP
  • Autoantibodies (e.g., ANCA, ANA, RF)
  • Complete blood count abnormalitie

Urinalysis: To check for kidney involvement
Imaging:

  • CT/MRI angiography to assess blood vessel inflammation
  • PET scans in large vessel vasculitis

Biopsy: Of affected tissue (e.g., skin, kidney, or temporal artery) to confirm inflammation and vessel wall damage

Conventional Treatment

Treatment depends on the type, severity, and organs affected. The goal is to reduce inflammation and prevent further damage.

  • Corticosteroids: Mainstay for initial control of inflammation
  • Immunosuppressive agents:
    • Cyclophosphamide for severe or life-threatening disease
    • Methotrexate, azathioprine, or mycophenolate for maintenance
  • Biologics:
    • Rituximab for ANCA-associated vasculitis
    • Tocilizumab for giant cell arteritis

Treatment usually involves an initial high-intensity phase to control active disease, followed by maintenance therapy to prevent relapse.

Stem Cell Therapy in Vasculitis

Stem cell therapy, particularly hematopoietic stem cell transplantation (HSCT), is being explored for refractory vasculitis cases.

Types of stem cells:

  • Hematopoietic stem cells (used in HSCT)
  • Mesenchymal stem cells (for their immunomodulatory effects)

Mechanisms:

  • Rebooting the immune system to eliminate autoreactive cells
  • Suppressing chronic inflammation
  • Promoting vascular and tissue repair

Clinical evidence:

  • Studies in severe, treatment-resistant vasculitis show promising outcomes, including long-term remission

Hyperbaric Oxygen Therapy (HBOT) in Vasculitis

HBOT may support vasculitis management, particularly for ischemic and ulcerative complications.

Mechanism:

  • Enhances oxygen delivery to inflamed and ischemic tissues
  • Reduces edema and inflammation
  • Stimulates angiogenesis and collagen synthesis
  • Supports healing of skin ulcers and wounds

Clinical applications:

  • Chronic non-healing wounds, especially in cutaneous vasculitis
  • Peripheral ischemia and gangrene prevention
  • May improve outcomes when used as adjunct to immunosuppression

Lifestyle and Supportive Care

Patients with vasculitis benefit from an integrated approach that includes:

  • Monitoring and follow-up: Regular assessments of organ function and treatment side effects
  • Infection prevention: Vaccinations and prophylaxis during immunosuppressive therapy
  • Diet and exercise: Anti-inflammatory diet and low-impact physical activity
  • Mental health support: Coping with chronic illness and medication side effects

Prognosis

The outlook for vasculitis varies based on the type, severity, and timeliness of treatment. With early and aggressive therapy, many patients achieve remission. However, relapses are common, and some may experience chronic organ damage. Advances in treatment, including biologics and regenerative medicine, are improving long-term outcomes.

Conclusion

Vasculitis is a complex autoimmune condition with potentially serious complications. While conventional therapies focus on controlling inflammation and preventing relapse, regenerative options like stem cell therapy and HBOT offer new avenues for managing refractory cases and promoting tissue repair. A multidisciplinary and personalized treatment strategy can help patients achieve remission, maintain organ function, and improve their quality of life.

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