Differentiating vasculitis from other diseases

Vasculitis
Overview
Classification Large-sized vessel vasculitis Takayasu's Arteritis Temporal Arteritis Medium-sized vessel vasculitis Kawasaki's Disease Polyarteritis Nodosa Small-sized vessel vasculitis Churg-Strauss Syndrome Cutaneous leukocytoclastic vasculitis Essential cryoglobulinemic vasculitis Henoch-Schonlein Purpura Microscopic polyangiitis Wegener's Granulomatosis Variable-sized vessel vasculitis Sjogren syndrome Cogan syndrome Single organ vasculitis Primary central nervous system angiitis
Causes
Differential Diagnosis

gsEditor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief:

Differential Diagnosis

Abbreviations: ABG= Arterial blood gas, ANP= Atrial natriuretic peptide, BNP= Brain natriuretic peptide, CBC= Complete blood count, COPD= Chronic obstructive pulmonary disease, CRP= C-reactive protein, CT= Computed tomography, CXR= Chest X-ray, DVT= Deep vein thrombosis, ESR= Erythrocyte sedimentation rate, HRCT= High Resolution CT, IgE= Immunoglobulin E, LDH= Lactate dehydrogenase, PCWP= Pulmonary capillary wedge pressure.

Diseases			Clinical manifestations								Para-clinical findings									Gold standard	Additional findings
			Symptoms					Physical examination
											Lab Findings				Imaging				Histopathology
			Headache	Fever	Weight loss	Arthralgia	Claudication	Bruit	HTN	Focal neurological disorder	Biomarker	CBC	ESR	Other	CT scan	Angiography	Ultrasound	Other
Large-Vessel Vasculitis	Takayasu arteritis		+	+/-	+	-	+	+	+/-	+/-	ESR	Leukocytosis, Anemia	↑	CRP	Aneurysmal dilatation of the aorta	Blood vessel stenosis	Circumferential thickening of the arterial wall (Macaroni sign)	PET-scan, Cardiac CT	Granulomatous inflammation of arteries	Arteriography	Coronary aneurysm
	Giant cell arteritis		+	-	+	+/-	-	-	-	+/-	ESR	Normal	↑	CRP	Stenosis, Occlusion, Dilatation	Aneurysm	-	Mural inflammation in MRA	Granulomatous inflammation of arteries	Biopsy	Jaw pain and claudication
	Neurological disease	Cerebral aneurysm	+	-	-	-	-	-	+/-	+	-	Normal	Normal	-	Well-defined round, slightly hyperattenuating lesion	Bulging out of the main lumen	-	Heterogeneous signal intensity in MRA	Layers of smooth muscle cells, Intact endothelium	Digital subtraction angiography (DSA)	Nausea, Vomiting
		Neurofibromatosis type 1	+/-	-	+/-	-	-	-	+	+	NF1 mutated gene	Normal	Normal	Neurofibromin gene	Bone abnormalities	Optical coherence tomography angiography (OCTA)	-	Optic nerve gliomas in MRI	Elongated spindle-shaped cells in neurofibromas	NIH diagnostic criteria	Cafe au Lait spot
		Neurofibromatosis type 2	+/-	-	+/-	-	+/-	-	-	+	NF2 mutated gene	Normal	Normal	Schwannomin	Meningioma, Schwannoma, Ependymoma	Fluorescein angiography showed retinal hamartoma	Localized schwannomas in nerve ultrasound	Schwannoma in MRI	Encapsulated biphasic nerve sheath tumor	NIH diagnostic criteria	Hearing loss, Vision loss
	Systemic disease	Fibromuscular dysplasia	+	-	+/-	+	+	+	+	+/-	Transforming growth factor β (TGF-β)	Normal	↑	↑ Cr or BUN	Alternating stenosis and dilatations in CT angiography	Stenosis in the renal arteries	Luminal narrowing alternating with dilatation (Beads sign)	Focal concentric, long-segment tubular stenosis or outpouching in MRA	Fibrodysplastic changes, Collagen deposition	Digital subtraction angiography (DSA)	Spontaneous coronary artery dissection (SCAD)
		Ehlers-Danlos syndrome	-	-	-	+	+/-	+/-	-	+/-	TGF-β	Normal	Normal	Cultured skin fibroblasts	Multiple vascular segments with aneurysms and dissections	Dissection of the posterolateral branch of the left circumflex coronary artery (LCx)	Visceral arteries abnormality	Vascular abnormalities in MRA	Thin and rare collagen bundles in the dermis	History and physical examination	Bleeding, Bruisability, Heart murmur
		Polymyalgia rheumatica (PMR)	+	-	+/-	+	-	-	-	-											Joint stiffness, Fatigue
		Amyloidosis	+/-	-	-	+/-	+/-	-	+	+
Diseases			Headache	Fever	Weight loss	Arthralgia	Claudication	Bruit	HTN	Focal neurological disorder	Biomarker	CBC	ESR	Other	CT scan	Angiography	Ultrasound	Other	Histopathology	Gold standard	Additional findings
Medium-Vessel Vasculitis	Polyarteritis nodosa
	Hepatitis B virus-associated polyarteritis nodosa
	Kawasaki disease
	Infectious disease	Parvovirus B19 infection
		Scarlet fever
		Toxic shock syndrome
		Mononucleosis
		Leptospirosis
		Lyme Disease
		Measles
		Rocky Mountain Spotted Fever
		Staphylococcal Scalded Skin Syndrome
		Toxic Epidermal Necrolysis
	Cardiovascular disease	Atrial Myxoma
		Cholesterol Embolism
		Segmental arterial mediolysis
	Systemic disease	Antiphospholipid Syndrome
		Juvenile Idiopathic Arthritis
Diseases			Headache	Fever	Weight loss	Arthralgia	Claudication	Bruit	HTN	Focal neurological disorder	Biomarker	CBC	ESR	Other	CT scan	Angiography	Ultrasound	Other	Histopathology	Gold standard	Additional findings
Small-Vessel Vasculitis	ANCA-associated vasculitis	Microscopic polyangiitis
		Granulomatosis with polyangiitis (Wegener’s)
		Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
		Hydralazine-associated ANCA-associated vasculitis
	Immune complex small-vessel vasculitis	Anti-glomerular basement membrane disease
		Cryoglobulinemic vasculitis
		Hepatitis C virus-associated cryoglobulinemic vasculitis
		IgA vasculitis (Henoch-Schönlein purpura)
		Hypocomplementemic urticarial vasculitis (anti-C1q vasculitis)
	Gastrointestinal disease	Acute mesenteric ischemia
	Cardiovascular disease	Infective Endocarditis
		Leukocytoclastic Vasculitis
	Pulmonary disease	Langerhans Cell Histiocytosis
		Lung Abscess
		Non-Small Cell Lung Cancer
		Small Cell Lung Cancer
		Asthma
		Pulmonary Infarction
	Renal disease	Acute Poststreptococcal Glomerulonephritis
	Hematologic disease	Hemolytic-Uremic Syndrome
		Chronic Lymphocytic Leukemia (CLL)
		Multiple Myeloma
		Hypereosinophilic Syndrome
		Non-Hodgkin Lymphoma
		Serum Sickness
		Disseminated Intravascular Coagulation
		Idiopathic Thrombocytopenic Purpura
		B cell lymphoproliferative disorder
	Systemic disease	Sarcoidosis
		Legionella Infection
		Systemic lupus erythematosus
		Rheumatoid arthritis
		Relapsing polychondritis
Diseases			Headache	Fever	Weight loss	Arthralgia	Claudication	Bruit	HTN	Focal neurological disorder	Biomarker	CBC	ESR	Other	CT scan	Angiography	Ultrasound	Other	Histopathology	Gold standard	Additional findings
Variable-vessel vasculitis	Behçet’s syndrome
	Cogan's syndrome
	Gastrointestinal disease	Inflammatory Bowel Disease
		Whipple's disease
		Sjögren's syndrome
Single-organ vasculitis	Primary central nervous system vasculitis
	Infectious disease	Aspergillosis
		Histoplasmosis
		Herpes Simplex Encephalitis
	Systemic disease	Eclampsia
		Fibromuscular Dysplasia