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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