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)	+	-	+/-	+	-	-	-	-	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
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		+	+	+	+	+	+	+/-	+/-	ESR, CRP	Leukocytosis, Normochromic anemia, Thrombocytosis	↑	↑ Cr or BUN, ↑ ALT or AST, Proteinuria	Focal regions of infarction or hemorrhage	Multiple microaneurysms, Hemorrhage due to focal rupture, Occlusion	Aneurysms and renal arteriovenous fistula in color Doppler sonography	-	Necrotizing inflammatory lesions	Angiography	Sudden weight loss, Abdominal pain
	Hepatitis B virus-associated polyarteritis nodosa		+/-	+/-	+	+	+/-	+/-	+	-	HBsAg	Leukocytosis, Normochromic anemia, Thrombocytosis	↑	↑ ALT or AST	Focal regions of infarction or hemorrhage	Microaneurysms in mesenteric artery	Aneurysms and renal arteriovenous fistula in color Doppler sonography	-	Necrotizing inflammatory lesions	Angiography	Peripheral neuropathy, Livedo reticularis
	Kawasaki disease		-	+	+/-	+	+	-	+/-	-	NT-proBNP, Meprin A, Filamin C	Normochromic anemia, ↑WBC with a left shift, Thrombocytosis	↑	Acute-phase reactants, ↓Cholesterol, ↓HDL, ↓ApolipoA	Coronary artery calcifications	Coronary artery aneurysms, stenosis or occlusion	Coronary artery anomaly in echocardiography	Electron beam CT (EBCT)	Acute destruction of the media by neutrophils, with loss of elastic fibers	History and physical examination	Diarrhea, Vomiting
	Infectious disease	Parvovirus B19 infection	+	+	+	+	-	-	-	+/-	B19 DNA, ↓Reticulocyte count	Anemia	↑	anti–parvovirus B19 IgM	-	-	Hydrops in fetal ultrasonography	-	-	B19 DNA	Purpuric rash, Erythema multiforme
		Scarlet fever	+	+	+/-	+	-	-	-	-	Antistreptolysin-O (ASO) titers	Leukocytosis	↑	↑CRP	Thickened pulmonary markings if pneumonia	-	-	-	Sparse neutrophilic perivascular infiltrate	History and physical examination	Sand-paper rashes, Sore throat
		Toxic shock syndrome	+	+	+	+	-	-	-	+/-	Procalcitonin	Leukocytosis with left shift	↑	Myoglobinuria, Sterile pyuria	Acute respiratory distress syndrome	-	-	-	Necrolysis of keratinocytes in epidermis, Perivascular lymphocytic infiltrate	Clinical criteria	Peeling or rashes, Organ dysfunction
		Mononucleosis	+	+	+	+	-	-	-	-	EBV DNA	Atypical lymphocyte	↑	Heterophile antibodies	CNS involvement	-	Splenomegaly	Encephalitis in MRI	Lymphoproliferative response in oropharynx, Lymphocytic infiltration in spleen	Heterophile antibody test	Splenomegaly, Palatal petechiae
		Leptospirosis	+	+	+	+	+/-	-	-	-	IL-6, IL-8 and IL-10	Anemia	-	↑Cr or BUN, ↑ALT or AST, Proteinuria	Diffuse alveolar hemorrhage	-	-	-	Toxin-mediated break down of endothelial cell membranes of capillaries	Culture and the microscopic agglutination test	Red eyes, Skin rash
		Lyme Disease	+/-	+	+/-	+	+/-	-	-	-	CXCL9 (MIG), CXCL10 (IP-10) and CCL19 (MIP3B)	Leukopenia, Thrombocytopenia	-	Microscopic hematuria, Proteinuria, ↑ALT or AST	Punctate lesions in periventricular white matter in brain SPECT	-	-	-	Acrodermatitis chronica atrophicans	Serologic tests	Erythema migrans
		Measles	+/-	+	+/-	+	-	-	-	-	Measles IgM	Leukopenia, Lymphocytosis, Thrombocytopenia	-	↑ALT or AST	Pneumonia	-	-	CXR	Spongiosis and vesiculation in the epidermis with scattered dyskeratotic keratinocytes	PCR	Generalized rash, Cough, Coryza, or Conjunctivitis
		Rocky Mountain Spotted Fever	+	+	+	+	-	-	-	-	R rickettsii serology	Thrombocytopenia, Anemia	-	↑ALT or AST, Hyponatremia	Infarction, edema, and meningeal enhancement	-	Myocardial or conduction abnormalities in echocardiography			Clinical criteria and tick exposure
		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	+/-	+/-	+	+	-	-	+	+/-				↓serum C4, Positive RF							Palpable purpura, Microscopic hematuria
		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