Vasculitis: Symptoms, Types, Causes and Treatment

Vasculitis is a complex group of disorders characterized by inflammation of blood vessels, leading to a wide range of symptoms and complications that can affect virtually every organ system. Understanding the symptoms, different types, underlying causes, and available treatments is essential for timely diagnosis and management. This article provides an in-depth, evidence-based overview for patients, caregivers, and healthcare professionals.

Symptoms of Vasculitis

Vasculitis can present with a broad and sometimes confusing array of symptoms, since it may affect blood vessels of any size in any organ of the body. Early recognition of these symptoms is crucial to prevent long-term complications.

Symptom	Description	Common Forms Affected	Sources
Purpura	Palpable red or purple skin spots	Small-vessel types	3 6 9 11
Fatigue	Persistent tiredness and malaise	All types	2 4 6
Fever	Low to high-grade, often unexplained	All types	2 4
Joint pain	Arthralgia or arthritis	IgA vasculitis, PAN	4 9
Organ damage	Kidney, lung, nervous system involvement	Systemic types	2 4 9 10

Table 1: Key Symptoms

Overview of Symptoms

Vasculitis symptoms vary widely depending on the type and organs involved. Some forms may only cause skin changes, while others can lead to life-threatening organ damage.

Skin Manifestations

• Palpable Purpura: The most classic sign, especially in small-vessel vasculitis, are raised red or purple spots that are usually found on the lower legs. These are due to inflammation and bleeding in the skin's blood vessels 3 6 9 11.
• Ulcers or Nodules: Skin ulcers and nodules may be seen, especially in more severe or chronic cases 6 11.

General (Constitutional) Symptoms

• Fatigue and Malaise: Many patients report feeling extremely tired, sometimes with low energy that does not improve with rest 2 4 6.
• Fever: Unexplained fever can be a sign of systemic inflammation. Persistent fevers may indicate more widespread involvement 2 4.

Joint and Muscle Involvement

• Arthralgia/Arthritis: Joint pain, swelling, and stiffness are common in several forms, particularly in IgA vasculitis and polyarteritis nodosa (PAN) 4 9.
• Muscle Pain: Aching muscles may also be present and contribute to general discomfort 5.

Organ-Specific Symptoms

• Kidney Involvement: Blood in urine, swelling, or kidney dysfunction can occur, especially in IgA vasculitis and ANCA-associated vasculitis 4 9.
• Lung Involvement: Shortness of breath, cough, and even bleeding in the lungs (hemoptysis) may be seen in several types, notably ANCA-associated and Behçet’s disease 2.
• Neurological Symptoms: Headaches, confusion, or even strokes can result from involvement of blood vessels in the brain (cerebral vasculitis) 10.

Types of Vasculitis

Vasculitis is not a single disease but a group of disorders, classified by the size of the blood vessels involved and sometimes by the underlying cause or associated features.

Type	Vessel Size/Target	Typical Age Group	Sources
Giant cell arteritis	Large arteries (temporal)	Older adults	1 4
Takayasu arteritis	Large arteries (aorta)	Young adults	1 4
Polyarteritis nodosa	Medium-sized arteries	Adults, children	1 4
Kawasaki disease	Medium arteries (esp. coronary)	Children	1 4 13
ANCA-associated vasculitis	Small vessels	Middle-aged/older	1 4 14
IgA vasculitis (HSP)	Small vessels (skin, kidney)	Children	4 7 9
Behçet’s disease	Variable	Young adults	1 4 8

Table 2: Main Types of Vasculitis

Classification by Vessel Size

• Large-Vessel Vasculitis: Includes giant cell arteritis (GCA) and Takayasu arteritis. GCA affects older adults, often causing headaches, scalp tenderness, and vision problems. Takayasu’s usually affects younger women and involves the aorta and its branches, leading to limb claudication and absent pulses 1 4.
• Medium-Vessel Vasculitis: Polyarteritis nodosa (PAN) and Kawasaki disease are the main examples. PAN can cause abdominal pain, kidney problems, and neurological symptoms. Kawasaki disease primarily affects children under five and may cause coronary artery aneurysms 1 4 13.
• Small-Vessel Vasculitis: This group includes IgA vasculitis (Henoch-Schönlein purpura), antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides such as granulomatosis with polyangiitis and microscopic polyangiitis, and cutaneous leukocytoclastic angiitis 1 3 4 9 14.

Variable and Other Forms

• Variable-Vessel Vasculitis: Behçet’s disease affects vessels of any size, causing recurrent oral/genital ulcers, skin lesions, and eye inflammation 1 4 8.
• Secondary Vasculitis: Can be associated with infections (like hepatitis B or C), autoimmune diseases (such as lupus or rheumatoid arthritis), drugs, or malignancies 1 5 6 11.

Special Cases

• Autoinflammatory Vasculitis: Rare monogenic diseases, such as Familial Mediterranean Fever and others, can present with distinctive vasculitic features 8.
• Cerebral Vasculitis: Primary angiitis of the central nervous system is a rare form affecting small and medium vessels in the brain 10.

Causes of Vasculitis

While some forms of vasculitis are idiopathic (unknown cause), many are linked to immune system dysfunction, infections, genetic factors, or environmental triggers.

Cause	Mechanism/Trigger	Notable Types	Sources
Autoimmune response	Immune attack on vessel walls	ANCA, GCA, PAN, SLE	1 2 4 8 14
Infections	Direct vessel invasion or immune complexes	Hepatitis B, HCV	5 12
Genetic factors	Inherited mutations/ethnic predisposition	FMF, Behçet’s, SAVI	4 8
Drugs and toxins	Hypersensitivity reactions	Secondary vasculitis	1 6 11
Environmental	Seasonal/infectious triggers	IgA vasculitis	7 9

Table 3: Main Causes of Vasculitis

Immune-Mediated Mechanisms

• Autoimmune Attack: Most primary vasculitides are autoimmune, where the body’s immune system mistakenly attacks its own blood vessel walls. In some cases, specific autoantibodies (like ANCA) are involved 1 2 14.
• Immune Complex Deposition: In diseases like IgA vasculitis, immune complexes form and deposit in vessel walls, activating inflammation and tissue damage 7 9.

Infection-Related Vasculitis

• Direct Infection: Certain viruses (e.g., hepatitis B and C) can directly infect or damage blood vessels, or trigger an abnormal immune response 5 12.
• Cryoglobulinemia: In hepatitis C, cryoglobulins—proteins that precipitate in the cold—can cause immune complex-mediated vasculitis 5.

Genetic and Environmental Factors

• Genetic Predisposition: Some forms are more common in specific ethnic groups or have a clear hereditary link, such as FMF and Behçet’s disease 4 8.
• Climate/Seasonal Triggers: Incidence of IgA vasculitis rises during fall and winter, implicating environmental or infectious triggers 7 9.

Drug-Induced and Secondary Vasculitis

• Medications and Toxins: Many drugs can cause vasculitis as a hypersensitivity reaction. These are usually reversible upon withdrawal of the offending agent 1 6 11.
• Secondary to Other Diseases: Vasculitis may also develop as a complication of autoimmune diseases (like lupus or rheumatoid arthritis), cancers, or paraneoplastic syndromes 1 6 11.

Treatment of Vasculitis

Vasculitis treatment is highly individualized, depending on the type, severity, and organs involved. The primary goals are to suppress inflammation, prevent complications, and address any underlying causes.

Therapy	Indication/Target	Example Drugs/Approach	Sources
Glucocorticoids	Most vasculitis types	Prednisone, methylprednisolone	12 13 14
Immunosuppressants	Severe/systemic disease	Cyclophosphamide, methotrexate, azathioprine, mycophenolate mofetil	10 11 12 13 14
Biologic agents	Refractory/targeted	Rituximab, anti-TNF drugs, avacopan	11 12 14
Antivirals	Infection-associated	Direct antivirals for HCV/HBV	5 12
Supportive care	Symptom management	NSAIDs, wound care, colchicine	3 6 11 12

Table 4: Main Treatments for Vasculitis

First-Line and Supportive Therapies

• Glucocorticoids: These are the cornerstone for most types of vasculitis, rapidly reducing inflammation. High doses may be used initially, followed by slow tapering 12 13 14.
• Supportive Measures: For mild or skin-limited disease, symptomatic treatments like NSAIDs, antihistamines, leg elevation, and wound care may be adequate 3 6 11 12.

Immunosuppressive and Biologic Therapies

• Immunosuppressants: Medications such as cyclophosphamide, methotrexate, azathioprine, and mycophenolate mofetil are often added for severe or organ-threatening disease and for maintenance therapy after remission is achieved 10 11 12 13.
• Biologic Agents: Newer drugs target specific immune pathways. Rituximab (anti-CD20) is used in ANCA-associated vasculitis. Avacopan, a C5a receptor inhibitor, has shown promise as a steroid-sparing agent, especially for ANCA-associated vasculitis 11 12 14.
• Other Biologics: Drugs like infliximab (anti-TNF), etanercept, and intravenous immunoglobulins are used in select cases or refractory disease 11 12.

Infection-Targeted Therapy

• Antivirals: Treating the underlying infection is paramount in hepatitis B or C-associated vasculitis. Direct-acting antivirals can lead to remission of cryoglobulinemic vasculitis 5 12.

Special Situations

• Plasmapheresis and IVIG: These may be considered in severe, refractory, or rapidly progressive disease (such as pulmonary hemorrhage, severe renal disease, or Kawasaki disease) 11 12.
• Surgical Intervention: Rarely needed but may be required for complications such as aneurysms or organ damage.

Treatment Considerations

• Monitoring and Side Effects: Long-term steroid and immunosuppressant use carries risks of infection, osteoporosis, diabetes, and other side effects. Newer, more targeted options aim to reduce toxicity 13 14.
• Tailoring Therapy: Treatment is individualized based on disease severity, organ involvement, and patient factors. Some forms (like IgA vasculitis in children) may resolve without intervention, while others require aggressive therapy 9 13.

Conclusion

Vasculitis is a diverse and often serious group of conditions that requires careful evaluation, diagnosis, and management. Understanding its symptoms, types, causes, and treatments is key to improving patient outcomes.

Main points covered:

• Vasculitis refers to inflammation of blood vessels, leading to symptoms like purpura, fatigue, fever, joint pain, and potential organ damage 2 3 4 9 11.
• Types are classified by vessel size (large, medium, small) and may be primary or secondary to other diseases 1 4.
• Causes include autoimmune responses, infections, genetics, environmental triggers, and drugs 1 4 5 7 8.
• Treatment is tailored to disease type and severity, involving steroids, immunosuppressants, biologics, antivirals, and supportive care 10 11 12 13 14.
• Advances in targeted therapy are improving outcomes and reducing treatment toxicity 12 13 14.

Early recognition and a multidisciplinary approach are essential for optimal management and better quality of life for those affected by vasculitis.