From the Guidelines
Vasculitis symptoms can vary greatly depending on the type and affected blood vessels, but typically include fever, fatigue, weight loss, muscle and joint pain, and a general feeling of illness, as noted in the 2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of giant cell arteritis and Takayasu arteritis 1.
Key Symptoms
The symptoms of vasculitis can be categorized based on the organs affected:
- Skin symptoms: rashes, purpura, or ulcers
- Respiratory symptoms: cough, shortness of breath, or sinus pain
- Neurological symptoms: numbness, weakness, or headaches
- Kidney involvement: blood or protein in urine
- Gastrointestinal symptoms: abdominal pain or bleeding
- Eye inflammation: redness, pain, or vision changes These symptoms result from inflammation of blood vessel walls, which causes vessel narrowing, reduced blood flow, and potential tissue damage, as described in the 2021 guideline 1.
Severity and Onset
The severity of vasculitis symptoms can range from mild to life-threatening, and may develop gradually or suddenly, as noted in the 2020 update of the EULAR recommendations for the management of large vessel vasculitis 1.
Importance of Early Diagnosis and Treatment
Anyone experiencing persistent unexplained symptoms, especially with fever, weight loss, or signs of organ dysfunction, should seek medical attention promptly, as early diagnosis and treatment significantly improve outcomes for vasculitis, as emphasized in the 2021 American College of Rheumatology/Vasculitis Foundation guideline 1 and the 2021 guideline published in Arthritis Care & Research 1.
Specific Symptoms of Giant Cell Arteritis and Takayasu Arteritis
Giant cell arteritis can present with cranial manifestations such as headaches, scalp tenderness, vision loss, and jaw claudication, as well as large vessel involvement resulting in arterial stenosis and aneurysms, causing absent pulses and limb claudication, as described in the 2021 guideline 1. Takayasu arteritis, on the other hand, causes granulomatous inflammation of the aorta and its branches, and is more common in younger women, with clinical manifestations including constitutional symptoms, elevated levels of inflammation markers, and arterial stenosis and/or aneurysms resulting in limb claudication and absent pulses, as noted in the 2021 guideline 1.
From the Research
Vasculitis Symptoms
- Vasculitis refers to a pathologic condition defined by inflammatory cell infiltration and destruction of blood vessels 2
- Systemic vasculitis is classified as primary or secondary, with neuropathy being a common complication of systemic vasculitis 2, 3
- Peripheral neuropathy may be the sole manifestation of vasculitis, a condition termed nonsystemic vasculitic neuropathy (NSVN) 2, 3
Clinical Features
- Most patients have clinical features of a subacute, progressive, generalized but asymmetric, painful, sensorimotor polyneuropathy 3
- Laboratory tests often indicate features of systemic inflammation, such as an elevated sedimentation rate or positive anti-neutrophil cytoplasmic antibody 3
- Electrodiagnostic evaluation shows multiple mononeuropathies or a confluent, asymmetric axonal neuropathy 3
Diagnosis
- The diagnosis of vasculitis should be established by tissue (preferably nerve) biopsy 2
- Nerve biopsy is necessary to establish the diagnosis in most cases, particularly in patients with NSVN 3
Treatment
- High-dose prednisone is the standard platform therapy for patients with systemic and NSVN 2, 3
- Cyclophosphamide is also used in the treatment of systemic vasculitis and NSVN, with intravenous pulse and daily oral cyclophosphamide probably offering similar efficacy 2, 4
- Other agents, such as azathioprine, methotrexate, intravenous immunoglobulin, mycophenolate mofetil, plasma exchange, and rituximab can be offered to patients who are intolerant or have a contraindication to cyclophosphamide 2, 3, 5