Differentiating Temporal arteritis from other diseases

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hamid Qazi, MD, BSc [2]

Overview

The differential of temporal arteritis include Takayasu arteritis, neurofibromatosis 1 and 2, fibromuscular dysplasia, Ehlers-Danlos syndrome, polymyalgia rheumatica (PMR), amyloidosis, and cerebral aneurysm.

Differentiating Temporal Arteritis from other Diseases

The following list is a differential diagnosis of disorders that temporal arteritis should be distinguished from based on headache:

• Takayasu arteritis
• Neurofibromatosis 1 and 2
• Fibromuscular dysplasia
• Ehlers-Danlos syndrome
• Polymyalgia rheumatica (PMR)
• Amyloidosis
• Cerebral aneurysm

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
	Giant cell arteritis		+	-	+	+/-	-	-	-	+/-	Pentraxin 3 (PTX3)	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)	+	-	+/-	+	-	-	-	-	Plasma fibrinogen	Normocytic, normochromic anemia	↑	CRP	Periodontoid localization of calcification	Vessel wall thickening, Increased mural contrast enhancement	Subacromial or subdeltoid bursitis	High F-FDG accumulation around the joints in FDG PET-CT	Small angular fibers, Pyknotic nuclear clumps, or target-targetoid fibers	-	Joint stiffness, Fatigue
		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