Variable-sized vessel vasculitis

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
Variable-vessel vasculitis	Behçet’s syndrome		-	-	-	+	-	-	+/-	-	CXCL1	Mild anemia, Neutrophilia	↑	Factor V Leiden	Focal CNS lesions	Aneurysm formation and thrombosis areas	Valve vegetations and ventricular thrombi in echocardiography	Meningoencephalitis  in MRI	Lymphocytic and plasma cell invasion in the prickle cell layer of the epidermis	Clinical criteria	Genital ulcerations, Oral ulceration
	Cogan's syndrome		+/-	-	-	+/-	-	-	-	+	Anti-Hsp70 antibodies	Anemia, Thrombocytosis	↑	RF, ANA	Thickening and enhancement of both posterior sclera	Stenosis, thrombosis or more lesions in aortic root	Aortic insufficiency in echocardiography	Early interstitial keratitis by slit lamp	Muscle necrosis and atrophy resembling myositis	-	Red eye, Hearing loss, Vertigo
	Gastrointestinal disease	Inflammatory Bowel Disease	+/-	+	+	+	+	-	-	-	Anti-Saccharomyces cerevisiae antibody (ASCA), P-ANCA	Leukocytosis, Anemia	↑	Iron or vitamin deficiency, Stool OB	Mesenteric fat stranding, bowel wall enhancement, increased vascularity (comb sign)	-	Fistulas, Abscesses, Stenosis	Grossly denuded mucosa with active bleeding in colonoscopy	Crypt abscesses and mucosal ulceration, Granulomas	Endoscopy	GI bleeding
		Whipple's disease	+	+/-	+	+	+	+	+	+	CCR6, Gut-homing marker integrin β7-chain, T whippelii DNA	Mild anemia, Neutrophilia	↑	72-hour fecal fat determination	Nonspecific malabsorption	-	Hepatosplenomegaly	-	Periodic acid-Schiff–positive macrophages infiltration in lamina propria of the small bowel	Broad-spectrum PCR amplifications	Cachexia, Glossitis
		Sjögren's syndrome	-	-	+/-	+	-	-	-	-	Anti-Ro and Anti-La, Anti-alpha-fodrin antibody	Anemia, Leukopenia, Eosinophilia	↑	Hypergammaglobulinemia, Low bicarbonate level, Hypokalemia	Salt and pepper or honeycomb appearance in parotid glands	-	Multicystic or reticular pattern in atrophic salivary gland	R/O obstructions or strictures with Sialography	Focal aggregates of lymphocytes	Schirmer test	Keratoconjunctivitis, Gingival inflammation