Systemic vasculitis is a group of rare autoimmune diseases characterised by inflammation of blood vessels throughout the body. These conditions can affect blood vessels of varying sizes, leading to various symptoms and potential complications. Understanding systemic vasculitis involves exploring its symptoms, causes, diagnosis, treatment, and impact on patients’ lives.
Symptoms of Systemic Vasculitis
Systemic vasculitis can manifest in various ways, depending on the type and location of blood vessel inflammation. Common symptoms include:
Constitutional Symptoms
- Fever – Low-grade fever without any apparent cause.
- Fatigue – A profound and persistent tiredness not relieved by rest.
Skin Symptoms
- Purpura – Small red or purple spots on the skin caused by bleeding under the skin.
- Petechiae – Tiny red or purple spots that appear in clusters.
Musculoskeletal Symptoms
- Arthralgia – Joint pain without swelling.
- Myalgia – Muscle pain and tenderness.
Neurological Symptoms
- Headaches – Severe and persistent headaches.
- Peripheral Neuropathy – Numbness, tingling, or weakness in the extremities.
Respiratory Symptoms
- Cough – Persistent cough, sometimes with blood-tinged sputum.
- Dyspnea – Shortness of breath, especially with exertion.
Renal Symptoms
- Hematuria – Blood in the urine, often accompanied by proteinuria.
- Renal Impairment – Decreased kidney function leads to fluid retention and electrolyte imbalance.
Gastrointestinal Symptoms
- Abdominal Pain – Persistent or recurrent abdominal pain, sometimes with gastrointestinal bleeding.
- Nausea and Vomiting – Especially after meals.
Causes and Risk Factors of Systemic Vasculitis
The exact cause of systemic vasculitis is unknown, but it is believed to result from genetic, environmental, and immunological factors.
Genetic Factors
- Family History – A family member with systemic vasculitis or another autoimmune disease may increase the risk.
- Genetic Predisposition – Certain genetic markers have been associated with an increased susceptibility to vasculitis.
Environmental Factors
- Infections – Some viral and bacterial infections have been implicated as triggers for vasculitis.
- Toxins – Exposure to certain chemicals and environmental toxins may increase the risk.
Immunological Factors
- Autoimmune Response – Abnormal activation of the immune system leads to inflammation and damage to blood vessels.
Diagnosis of Systemic Vasculitis
Diagnosing systemic vasculitis requires clinical evaluation, laboratory tests, imaging studies, and, sometimes, tissue biopsy.
Clinical Evaluation
- Medical History – Detailed questions about symptoms, their onset, duration, and progression.
- Physical Examination – Assessment of skin changes, joint involvement, and organ function.
Laboratory Tests
- Complete Blood Count (CBC) – To assess for anaemia, leukocytosis, and thrombocytosis.
- Inflammatory Markers – Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) may be elevated.
- Autoantibodies – Detection of specific autoantibodies such as antineutrophil cytoplasmic antibodies (ANCA) and antinuclear antibodies (ANA).
Imaging Studies
- Chest X-ray or CT Scan – To evaluate lung involvement and detect pulmonary nodules or infiltrates.
- Renal Ultrasound – To assess kidney size, structure, and blood flow.
Tissue Biopsy
- Skin Biopsy – To examine affected blood vessels and surrounding tissues for signs of inflammation and damage.
- Renal Biopsy – In cases of suspected renal involvement, to assess the extent of kidney damage.
Systemic Vasculitis Treatment
Treatment for systemic vasculitis aims to suppress inflammation, relieve symptoms, prevent organ damage, and induce and maintain remission. A multidisciplinary approach involving rheumatologists, nephrologists, pulmonologists, and other specialists is often required.
Medications
- Glucocorticoids – High-dose corticosteroids to induce remission.
- Immunosuppressants – These drugs are used to suppress the immune response and reduce inflammation.
- Biologics – Targeted therapies such as TNF or interleukin inhibitors for refractory cases.
Symptomatic Treatment
- Pain Management – Analgesics and nonsteroidal anti-inflammatory drugs (NSAIDs) for joint and muscle pain.
- Antihypertensives – To control blood pressure and prevent complications of renal involvement.
- Anticoagulants – In cases of thrombotic vasculitis, they are used to prevent blood clots.
Supportive Therapy
- Plasmapheresis – To remove circulating immune complexes and antibodies in severe cases.
- Intravenous Immunoglobulin (IVIG) – To modulate the immune response and reduce inflammation.
- Dialysis – In cases of renal failure, renal replacement therapy is required.
Systemic vasculitis is a complex group of autoimmune diseases that require early diagnosis, prompt treatment, and ongoing monitoring to prevent complications and improve outcomes. While there is no cure, advancements in research and treatment offer hope for better management and improved quality of life for those living with systemic vasculitis. Education, support, and access to specialised care are crucial for optimising patient outcomes and enhancing overall well-being.
