From the Guidelines
Vasculitis is primarily precipitated by immune system dysfunction, where the body's immune response mistakenly attacks blood vessel walls, and can be triggered by several factors including infections, autoimmune disorders, certain medications, environmental exposures, and genetic predisposition, as supported by the most recent evidence from 1.
Key Factors
- Infections such as hepatitis B and C viruses, streptococcal bacteria
- Autoimmune disorders like rheumatoid arthritis, lupus
- Certain medications including hydralazine, propylthiouracil, cocaine adulterated with levamisole, and minocycline, as noted in 1
- Environmental exposures such as silica dust, certain chemicals
- Genetic predisposition
Pathophysiology
The pathophysiology involves immune complex deposition in vessel walls, antineutrophil cytoplasmic antibodies (ANCA) attacking neutrophils, or direct T-cell mediated damage, leading to inflammation of blood vessels, causing vessel wall damage, narrowing, weakening, or scarring that disrupts blood flow and can damage organs, as discussed in 1.
Clinical Considerations
In many cases, vasculitis is idiopathic, meaning the specific trigger cannot be identified despite the clear inflammatory process occurring in the blood vessels. The diagnosis and management of vasculitis require a comprehensive approach, including clinical assessment, laboratory tests, and imaging studies, as recommended in 1 and 1.
Management
The management of vasculitis involves immunosuppressive therapy, including cyclophosphamide and rituximab, as well as plasma exchange in certain cases, as supported by the evidence from 1.
Important Considerations
It is essential to consider the potential side effects of immunosuppressive therapy, such as the risk of bladder cancer associated with cyclophosphamide use, as noted in 1.
Recent Guidelines
Recent guidelines, such as those from the European League Against Rheumatism (EULAR) and the European Renal Association, provide recommendations for the management of ANCA-associated vasculitis, emphasizing the importance of shared decision-making between clinicians and patients, as discussed in 1.
From the FDA Drug Label
Cases of vasculitis resulting in severe complications and death have been reported in patients receiving propylthiouracil therapy The cases of vasculitis include: glomerulonephritis, leukocytoclastic cutaneous vasculitis, alveolar/pulmonary hemorrhage, cerebral angiitis, and ischemic colitis. Most cases were associated with anti-neutrophilic cytoplasmic antibodies (ANCA)-positive vasculitis
Vasculitis is precipitated by propylthiouracil therapy, with most cases associated with ANCA-positive vasculitis. Key types of vasculitis include:
- Glomerulonephritis
- Leukocytoclastic cutaneous vasculitis
- Alveolar/pulmonary hemorrhage
- Cerebral angiitis
- Ischemic colitis 2
From the Research
Precipitating Factors of Vasculitis
The following are some of the factors that can precipitate vasculitis:
- Infections, particularly hepatitis B and C, and haemorrhagic fever 3, 4, 5, 6, 7
- Cancer 3
- Autoimmune diseases such as rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), and Sjogren's 3
- Drugs or allergic reactions 3
- Viral and other microbial agents, including Staphylococcus spp, Streptococcus spp, Salmonella spp, Treponema spp, Rickettsia spp, Cytomegalovirus, Herpes Simplex Virus 1 and 2, and others 5, 6
Mechanisms of Vasculitis
Vasculitis can occur through different mechanisms, including:
- Direct infection of the vascular wall, leading to destruction and inflammation 5, 6
- Indirect infection, where the immune system reacts against the blood vessels, leading to inflammation 5, 6
- Immune complex-mediated reaction, cell-mediated hypersensitivity, or inflammation due to immune dysregulation triggered by bacterial toxin and/or superantigen production 6
Types of Vasculitis
There are different types of vasculitis, including: