From the Guidelines
Vasculitis is a group of disorders characterized by inflammation of blood vessels, which can lead to damage and narrowing of the vessels, potentially restricting blood flow to organs and tissues, as described in the most recent guideline by KDIGO in 2024 1.
Definition and Characteristics
Vasculitis can occur as a primary disease or secondary to another condition like an infection, medication reaction, or autoimmune disorder. The symptoms vary widely depending on which blood vessels are affected and may include fever, fatigue, weight loss, muscle and joint pain, rash, nerve problems, or organ dysfunction.
Diagnosis and Treatment
Treatment typically involves corticosteroids like prednisone to reduce inflammation, often combined with immunosuppressive medications such as cyclophosphamide, methotrexate, or rituximab depending on the severity and type of vasculitis, as recommended by EULAR in 2009 1.
Key Considerations
The duration of treatment can range from months to years, with careful monitoring for side effects. Early diagnosis and treatment are crucial to prevent permanent damage to affected organs. The underlying cause of most forms of vasculitis involves the immune system mistakenly attacking blood vessel walls, though the exact triggers remain unclear in many cases.
- The clinical manifestations associated with vasculitis include microscopic hematuria with dysmorphic red blood cells and red cell casts, and proteinuria that is usually moderate, as noted in the KDIGO 2024 guideline 1.
- A positive test for myeloperoxidase (MPO)- or proteinase 3 (PR3)-ANCA supports a diagnosis of ANCA-associated vasculitis, as stated in the EULAR recommendations 1.
- The need to treat extrarenal vasculitis may influence treatment choices for kidney vasculitis, highlighting the importance of a comprehensive approach to management, as emphasized in the KDIGO 2024 guideline 1.
From the Research
Definition of Vasculitis
- Vasculitis is an autoimmune connective tissue disease that targets blood vessels in the body, leading to inflammation and potential damage to vital organs 2.
- It is characterized by acute kidney injury, haematuria, and proteinuria, and can present as rapidly progressive glomerulonephritis 3, 4.
Types of Vasculitis
- Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a type of vasculitis that can present with life-threatening lung-kidney syndromes 5.
- Renal vasculitis is a type of vasculitis that affects the kidneys and can cause acute kidney failure, haematuria, and proteinuria 3, 4.
Treatment of Vasculitis
- Treatment of vasculitis typically involves the use of steroid and non-steroid agents, with or without adjunctive plasma exchange 3, 4.
- Combination therapy with rituximab, low-dose cyclophosphamide, and plasma exchange has been shown to be effective in inducing remission in patients with severe ANCA-associated vasculitis 5, 6.
- Plasma exchange has been shown to significantly reduce the risk of end-stage kidney disease in patients with renal vasculitis 3, 4.