Single organ 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
Single-organ vasculitis	Primary central nervous system vasculitis		+	+	+	-	+	-	+	+	von Willebrand factor antigen (vWF)	Normal	-	CSF pleocytosis, predominantly lymphocytes	Cerebral infarcts or hemorrhages with mass effect, or hydrocephalus	Aneurysm in circle of Willis	-	Progression of the disease or response to therapy in MRI	Chronic granulomatous inflammation and giant cells	Histological confirmation	Skin rash, Purpura
	Infectious disease	Aspergillosis	+	+	+	-	-	-	-	+	Aspergillus nucleic acid in blood, Galactomannan	Eosinophilia	↑	↑Serum IgE	Aspergilloma mass within a cavity	Mass effect stenosis	-	Aspergilloma mass within the brain in MRI	Septate hyphae, branching at acute angles, and tissue necrosis with granulomata and blood vessel invasion	Histological confirmation	Hemoptysis, Aspergilloma
		Histoplasmosis	+	+	+	+	+	-	-	+	-	Mild anemia	↑	↑ALP, ↑LDH	Cerebral histoplasmosis	-	Valvular involvement in echocardiography	PFT	Presence of yeast forms in tissue through hematoxylin and eosin staining	Sputum cultures	Pneumonia, Mediastinitis
		Herpes Simplex Encephalitis	+	+	-	-	-	-	-	+	HSV DNA	Mild lymphocytosis	↑	CSF pleocytosis	Low-density lesions in the temporal and/or frontal lobe	-	-	Hemorrhagic lesion in white matter	Multinuclear giant cells	PCR or brain biopsy	Seizures, Vomiting
	Systemic disease	Eclampsia	+	+	-	+	-	-	+	+	VEGF, PlGF, Soluble FLT-1	Anemia, Thrombocytopenia, Schistocytes	-	↑Bilirubin, ↓Haptoglobin, ↑LDH, ↑Cr	Cortical hypodense areas in the occipital lobes, Diffuse cerebral edema	-	Poor fetal growth, Oligohydramnios, Abnormal umbilical artery	Increased signal at the gray-white matter junction in MRI	-	24-hour urine study	Seizure, Edema
		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)