From the Guidelines
Systemic vasculitis presents with a range of symptoms that vary depending on which blood vessels and organs are affected, including fever, fatigue, weight loss, muscle and joint pain, and night sweats, as well as organ-specific symptoms such as skin manifestations, respiratory symptoms, kidney involvement, neurological symptoms, gastrointestinal symptoms, cardiovascular manifestations, and eye involvement 1. The symptoms of systemic vasculitis can be diverse and depend on the specific type of vasculitis and the organs involved. Common symptoms include:
- Fever, fatigue, weight loss, muscle and joint pain, and night sweats
- Skin manifestations such as rashes, purpura (purple spots), or ulcers
- Respiratory symptoms such as cough, shortness of breath, or bloody sputum
- Kidney involvement causing hematuria, proteinuria, and decreased kidney function
- Neurological symptoms ranging from headaches and confusion to nerve damage causing numbness or weakness
- Gastrointestinal symptoms including abdominal pain, bleeding, or diarrhea
- Cardiovascular manifestations such as chest pain, heart failure, or hypertension
- Eye involvement causing redness, pain, vision changes, or even blindness According to the 2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of polyarteritis nodosa, patients with systemic vasculitis frequently present with systemic symptoms such as fever and weight loss, as well as neurologic, cutaneous, renal, and gastrointestinal manifestations 1. Early recognition of these symptoms is crucial as prompt treatment with immunosuppressive medications can prevent permanent organ damage and improve outcomes 1. The severity and combination of symptoms depend on the specific type of vasculitis, with some forms affecting predominantly small vessels (like granulomatosis with polyangiitis) and others affecting larger vessels (like giant cell arteritis) 1. In terms of diagnosis, tissue biopsy of an affected organ or angiography can confirm the diagnosis of systemic vasculitis, with typical histologic findings including mixed-cell inflammatory infiltrates in the vessel wall and fibrinoid necrosis, and angiography findings including saccular or fusiform aneurysms and stenotic lesions in the affected arteries 1.
From the Research
Symptoms of Systemic Vasculitis
- Systemic vasculitis can cause a range of symptoms, including peripheral neuropathy, which is a well-recognized consequence of the condition due to peripheral nerve infarction with Wallerian degeneration 2
- The symptoms of vasculitic neuropathy can include a subacute, progressive, generalized but asymmetric, painful, sensorimotor polyneuropathy 2
- Laboratory tests may indicate features of systemic inflammation, such as an elevated sedimentation rate or positive anti-neutrophil cytoplasmic antibody 2
- Electrodiagnostic evaluation may show multiple mononeuropathies or a confluent, asymmetric axonal neuropathy 2
Classification of Systemic Vasculitis
- Systemic vasculitis can be classified as primary or secondary, with primary vasculitis including conditions such as polyarteritis nodosa, Churg-Strauss syndrome, and Wegener granulomatous 2, 3
- Secondary vasculitis can occur as a complication of a connective tissue disorder, infection, medication, or malignancy 2, 3
Diagnosis of Systemic Vasculitis
- The diagnosis of vasculitic neuropathy is straightforward in patients with an established diagnosis of systemic vasculitis and classic features of mononeuritis multiplex 2
- Nerve biopsy is necessary to establish the diagnosis in most cases, particularly in patients with nonsystemic vasculitic neuropathy (NSVN) 2, 3