Vasculitis
Clinic
- Vasculitis is a group of disorders that destroy blood vessels by inflammation. Three site:
- Lymphangitis: lymphatic vessels
- Phlebitis: veins
- Arteritis: arteries
Necrotizing vasculitis, also called systemic necrotizing vasculitus, is a category of vasculitis, comprising vasculitides that present with necrosis.
Examples include giant cell arteritis, microscopic polyangiitis, and granulomatosis with polyangiitis.
Types
Large vessel vasculitis
Medium vessel vasculitis
- Polyarteritis nodosa (PAN): Systemic necrotizing vasculitis and aneurysm formation affecting both medium and small arteries.
- If only small vessels are affected, it is called microscopic polyangiitis, although it is more associated with granulomatosis with polyangiitis than to classic PAN.
- Kawasaki disease
- Buerger's disease
Small vessel vasculitis
- Behçet's syndrome
- Eosinophilic granulomatosis with polyangiitis
- Cutaneous vasculitis
- Granulomatosis with polyangiitis
- Henoch–Schönlein purpura
- Microscopic polyangiitis
Pauci-immune
Granulomatosis with polyangiitis.
Eosinophilic granulomatosis with polyangiitis
Immune complex
Hypersensitivity vasculitis (allergic vasculitis)
IgA vasculitis (formerly known as Henoch–Schonlein purpura).
Essential cryoglobulinemic vasculitis
Secondary vasculitis
- SLE
- RA
- Relapsing polychondritis
- Behçet's disease
- Other connective tissue disorders
Miasms
HBV
HCV
CMV
EBV
B19
Signs and symptoms[edit]
Patients usually present with systemic symptoms with single or multiorgan dysfunction. Common (and nonspecific) complaints include fatigue, weakness, fever, joint pains, abdominal pain, hypertension, chronic kidney disease, and neurologic dysfunction. The following symptoms should raise a strong suspicion of a vasculitis:
- Mononeuritis multiplex. Also known as asymmetric polyneuropathy, in a non-diabetic this is suggestive of vasculitis.
- Palpable purpura. If patients have this in isolation, it is most likely due to cutaneous leukocytoclastic vasculitis. If the purpura is in combination with systemic organ involvement, it is most likely to be Henoch–Schönlein purpura or microscopic polyangiitis.
- Pulmonary-renal syndrome. Individuals who are coughing up blood and have kidney involvement are likely to have granulomatosis with polyangiitis, microscopic polyangiitis, or anti-GBM disease (Goodpasture syndrome).
Signs / Symptoms
- General symptoms: Fever, unintentional weight loss
- Skin: Palpable purpura, livedo reticularis
- Myalgia, Arthralgia, Arthritis
- Mononeuritis multiplex, headache, stroke, tinnitus, reduced visual acuity, acute visual loss
- Heart and arteries: Heart attack, high blood pressure, gangrene
- Respiratory tract: Nosebleeds, bloody cough, lung infiltrates
- GI tract: Abdominal pain, bloody stool, perforations (hole in the GI tract)
- Glomerulonephritis
Some types
- Syphilitic aortitis is infectious (aortitis simply refers to inflammation of the aorta, which is an artery.)
- Large vessel: Takayasu's arteritis, Temporal arteritis
- Medium vessel:
| Comparison of major types of vasculitis | ||
|---|---|---|
| Vasculitis | Affected organs | Histopathology |
| Cutaneous small-vessel vasculitis | Skin, kidneys | Neutrophils, fibrinoid necrosis |
| Granulomatosis with polyangiitis | Nose, lungs, kidneys | Neutrophils, giant cells |
| Eosinophilic granulomatosis with polyangiitis | Lungs, kidneys, heart, skin | Histiocytes, eosinophils |
| Behçet's disease | Commonly sinuses, brain, eyes and skin; can affect other organs such as lungs, kidneys, joints | Lymphocytes, macrophages, neutrophils |
| Kawasaki disease | Skin, heart, mouth, eyes | Lymphocytes, endothelial necrosis |
| Buerger's disease | Leg arteries and veins (gangrene) | Neutrophils, granulomas |
| "Limited" granulomatosis with polyangiitis vasculitis | Commonly sinuses, brain, and skin; can affect other organs such as lungs, kidneys, joints; | |