Vasculitis - Differential Diagnosis Framework
When to Consider Vasculitis:
• Purpura, ischemic skin lesions
• Mononeuritis multiplex
• Hematuria, proteinuria, Rapidly progressing GN
• New pulmonary and renal abnormalities
Vasculitis Differential Diagnosis:
• Systemic rheumatic diseases, such as SLE, atherosclerotic disease, drug reactions, and vaso-occlusive processes. Among the most important diseases to exclude are infections and malignancies.
Presentation:
Systemic Symptoms (SX) in Vasculitis:
• Fevers
• Fatigue
• Anorexia
• Weight loss
• Night sweats
• Arthralgias
• Eye inflammation, particularly scleritis
• Palpable purpura: Strong sign of cutaneous leukocytoclastic vasculitis
• Sensory and/or motor neuropathy
• Absent, diminished, or tender pulses, bruits, or blood pressure discrepancies
LARGE VESSEL VASCULITIS (GRANULOMATOUS DISEASE):
Takayasu:
• Age < 30 years
• Aorta and branches
• Fever, malaise, weight loss, arthralgia, arm/leg claudication, pulse deficits vascular bruits, asymmetric arm BP readings, carotodynia
• TIA, stroke, and mesenteric ischemia
• Elevated ESR and CRP
GCA (Giant Cell Arteritis):
• Aorta and Superficial temporal artery
• Age > 50 years
• New headache, scalp tenderness, jaw claudication, visual disturbance
• Temp. artery tenderness - decreased pulsation/tenderness
• ESR > 50mm/hr
• Biopsy: Granulomatous inflammation/Multinucleated giant cells
PMR (Polymyalgia Rheumatica):
• Aching, morning stiffness in proximal muscles of shoulder and hip
• Muscle strength & enzymes normal
• May develop in pts with GCA or as primary condition
MEDIUM VESSEL VASCULITIS:
Polyarteritis Nodosa:
• PAN most commonly affects the skin, neurologic, and musculoskeletal systems
• Kidneys: Non-glomerular ischemia
• Skin: Erythematous nodules, purpura, livedo reticularis, ulcers, and bullous or vesicular eruption
• Joints: Myalgia and muscle weakness
• Nerves: Neuropathy, mononeuritis multiplex
• GI tract: Abdominal pain
• HTN
• Check: HBV
Kawasaki Disease:
• Mucocutaneous lymph node syndrome
• Coronary arteries
• Fever
• Conjunctivitis, mucositis
• Rash, arthritis
Primary Angiitis of the Central Nervous System:
• Onset is 50 years
• Progressive symptoms of headache, cognitive impairment, neurologic deficits, TIA and strokes
• Cerebrospinal fluid (CSF) is abnormal in 90%, with elevated protein, lymphocytic pleocytosis, and occasional oligoclonal bands
• MRI shows nonspecific white and gray matter changes and infarcts
SMALL VESSEL VASCULITIS:
ANCA Associated / Pauci Immune (ANCA specific for myeloperoxidase (MPO-ANCA) or proteinase 3 (PR3-ANCA)):
Microscopic polyangiitis
• No Granulomas (ANCA is present in > 30 / MPO > PR3)
• Pulmonary capillaritis, palpable purpura
• Pauci immune GN (rapidly progressive)
EGPA (Eosinophilic Granulomatosis with Polyangiitis):
• Eosinophilia, asthma, chronic rhinosinusitis
• Granulomas, IgE, pulmonary infiltrates, hemoptysis, MPO > PR3 60/
GPA (Granulomatosis with Polyangiitis):
• (-Granulomas) ANCA is present in > 80 percent
• (80 to 90 percent have PR3-ANCA)
• Necrotizing, pauci-immune glomerulonephritis
• Granulomatous inflammation of the upper and lower respiratory tracts
• Recurrent middle ear infections, destructive rhinitis/sinusitis, saddle nose, tracheal collapse, pulmonary infiltrates/cavities, hemoptysis, pauci-immune GN
Immune Complex Vasculitis (Glomerulonephritis is often present)
Henoch Schonlein Purpura (IgA Vasculitis):
• Affects the skin and gastrointestinal tract, and often causes arthritis. Ig A nephropathy, IgA (cmpkx deposits skin)
• Palpable purpura, joint & gut involvement, abdominal pain and GN
Anti-GBM (10 - 40 Y / ANCA + MPO)
Cryoglobulinemic Vasculitis:
• Skin lesions (red macules, palpable purpura, nodules or ulcers)
• GN, Mono Multiplex, Elevated AST ALT
• Hypocomplementemia: Urticarial Vasculitis (anti-C1q Vasculitis)
• Hypocomplementemic Urticarial Vasculitis (anti-C1q Vasculitis)
• GN, Mononeuropathy, Glomerulonephritis, arthritis, obstructive pulmonary disease, and ocular inflammation
Hypersensitivity Vasculitis (Leukocytoclastic Vasculitis)
• Palpable purpura (lower legs), cutaneous vesicles, pustules, maculopapular lesions, urticaria, recent viral infection, drug exposure or Dx of malignancy
• Variable-vessel vasculitis
• Behcet syndrome: Recurrent oral and/or genital aphthous ulcers, cutaneous, ocular, articular, gastrointestinal, and/or central nervous system involvement. Thrombosis and arterial aneurysms can also occur. Uveitis, chorioretinitis, CNS large vessel vasculitis
• Cogan syndrome: Ocular inflammatory lesions - interstitial keratitis, uveitis, and episcleritis as well as inner ear disease
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