Large-sized vessel vasculitis with claudication

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Eiman Ghaffarpasand, M.D. [2]

Abbreviations: ABG= Arterial blood gas, ANA= Antinuclear antibody, ANP= Atrial natriuretic peptide, ASO= Antistreptolysin O antibody, 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, PCR= Polymerase chain reaction, PFT= Pulmonary function test.

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/ Echocardiography	Other
Large-Vessel Vasculitis	Takayasu arteritis		+	+/-	+	-	+	+	+/-	+/-	MMP-3 and MMP-9	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
	Neurological disease	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
		Amyloidosis	+/-	-	-	+/-	+/-	-	+	+	Immunoglobulin light chain(Amyloid)	Anemia	Normal	↑ Cr or BUN, ↑ ALT or AST	Diffusely hypoattenuating and enlarged liver	Amyloid deposition in the media and adventitia of small arteries	Solid organs increased echogenicity	Tc-DPD for cardiac amyloid deposits	Extracellular deposition of fibrillar proteins	Biopsy	Cardiomegaly, Dyspnea