Small-sized vessel vasculitis with hypertension

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
Small-Vessel Vasculitis	Immune complex small-vessel vasculitis	Anti-glomerular basement membrane disease	+	+/-	-	-	-	-	+	-	Anti-GBM antibodies	Hypochromic microcytic anemia, Thrombocytopenia	-	↓C3 level	Pulmonary hemorrhage	-	Normal kidneys	Alveolar infiltrates spreading from the hilum in CXR	Cellular crescents in the glomeruli, Intra-alveolar hemorrhages	Anti-GBM antibodies	Hemoptysis, Hematuria
		Hepatitis C virus-associated cryoglobulinemic vasculitis	+/-	+/-	+	+	-	-	+	+/-	HCV RNA, Cryoglobulins	Leukocytosis, Anemia	↑	↓Serum C4, Positive RF	Increased hepatic echogenicity	-	Hepatomegaly, Splenomegaly	Increased hepatic echogenicity in MRI	Vasculitic skin, Antigen infilteration in lesions	HCV RNA, Histological confirmation	Palpable purpura, Microscopic hematuria
	Pulmonary disease	Pulmonary Infarction	+/-	-	-	-	-	-	+	-	D-dimer	Mild leukocytosis, Mild anemia	-	Hypoxemia, Hypocarbia or Hypercarbia, Respiratory alkalosis	Pulmonary embolism	Low-density filling defect within the pulmonary artery	Pericardial effusion in echocardiography	Pulmonary infiltrates, atelectasis, and pleural effusions in CXR	Infarct induced apoptosis	Pulmonary artery angiography	Cough, Hemoptysis
	Renal disease	Acute Poststreptococcal Glomerulonephritis	-	-	-	+	-	-	+	+/-	Antistreptolysin-O (ASO) titers	Leukocytosis	↑	Hypocomplementemia	-	-	Normal to slightly enlarged kidneys	Central venous congestion in a hilar pattern in CXR	Hypercellularity of endothelial and mesangial cells, Infiltration of the glomerular tuft with polymorphonuclear cells	Histological confirmation	Hematuria
	Hematologic disease	Hemolytic-Uremic Syndrome	+	+	-	+	-	-	+	+	C5b-9, ADAMTS13	Anemia, Thrombocytopenia, Reticulocytosis	↑	↑Lactate dehydrogenase (LDH), Hypercalcemia	Thalami, brainstem, or cerebellum abnormality	Cerebral microangiopathy or hypertension	Hypoechoic kidney	Abnormal hyperintensity in the brain cisterns in MRI	Microthromboses include fibrin thrombi that may occlude the glomerular tuft	Clinical findings coupled with laboratory abnormalities	Hematuria, Proteinuria
		Multiple Myeloma	+	-	+	+	+	+	+/-	+/-	Ig light chain	Anemia, Thrombocytopenia, Leukopenia	↑	Bone marrow aspiration and biopsy, ↑Cr	Osseous involvement and lytic lesions	Peripheral zone of increased vascularity in lesions	-	Punched-out lesion in skull X-ray	Clonal proliferation of plasma cells	Protein electrophoresis plus conventional X-rays	Constipation
		Non-Hodgkin Lymphoma	+	+	+	+	+/-	+/-	+/-	+/-	MYC, BCL2, BCL6, and TP53	Lymphocytosis, Anemia, Thrombocytopenia	↑	↑Lactate dehydrogenase (LDH), Hypercalcemia	Enlarged lymph nodes, Hepatosplenomegaly, Filling defects in the liver and spleen	-	Hepatosplenomegaly	Mediastinal lymphadenopathy	Small cleaved or noncleaved, intermediate, or large cell with a follicular or diffuse pattern	Surgically excised tissue biopsy	Easy bruising, Testicular mass, Skin lesion
		Disseminated Intravascular Coagulation	+/-	+	-	+/-	-	-	+	+	Fibrin degradation product (FDP)	Thrombocytopenia, Schistocytes	↑	↑D-dimer, aPTT and PT	Intracranial hemorrhage	-	-	-	Ischemia and necrosis due to fibrin deposition in small and medium-sized vessels	Clinical findings coupled with laboratory abnormalities	Acral cyanosis, Hemorrhagic skin infarctions
	Systemic disease	Systemic lupus erythematosus	+	+	+	+	-	-	+	+	Anti dsDNA, ANA	Leukopenia, Lymphopenia, Anemia, Thrombocytopenia	↑	↑Cr or BUN, ↑ALT or AST, Proteinuria	Interstitial lung disease, Pneumonitis, Pulmonary emboli, Alveolar hemorrhage	Aneurysms	Pericardial effusion, pulmonary hypertension, or verrucous Libman-Sacks endocarditis in echocardiography	Central nervous system (CNS) lupus white-matter changes in MRI	Staging lupus nephritis	Anti-dsDNA antibody test	Skin rashes or photosensitivity