Small-sized vessel vasculitis with claudication

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	Cryoglobulinemic vasculitis	+/-	+/-	-	+	+/-	-	-	-	C4 component	Leukocytosis, Anemia	↑	ANA, hypocomplementemia	R/O underlying malignancy	Stenosis or occlusions of the visceral arteries	Bacterial endocarditis in echocardiography	Interstitial involvement or pleural effusions in CXR	HCV-associated proteins in vasculitic skin, Intraluminal cryoglobulin deposits	Histological confirmation	Acrocyanosis, Retinal hemorrhage, Purpura
	Hematologic disease	Chronic Lymphocytic Leukemia (CLL)	+	+	+	+	+/-	-	-	+/-	CD5, CD19, CD20, IgVH	Absolute lymphocytosis, Smudge cells	↑	Flow cytometry	Staging	-	-	-	Large atypical cells, cleaved cells, and prolymphocytes	Chromosomal and genetic testing	Easy bruising
		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
		Serum Sickness	+	+	-	+/-	+/-	-	-	+/-	IL-1, IL-6, TNF	Leukopenia	↑	Polyclonal gammopathy, ↑Cr, Cryoglobulinemia	-	-	-	-	Arteritic lesions are focal, necrotizing, and inflammatory involving all layers of the artery	Clinical findings coupled with laboratory abnormalities	Hematuria, Skin rash
	Systemic disease	Rheumatoid arthritis	-	-	+	+	+	+	-	-	RF, Anti-CCP antibody	Anemia	↑	↑Cr or BUN, ↑ALT or AST, ANA	Microfractures	Aneurysms	Effusions in joints	Basilar invagination with cranial migration of an eroded odontoid peg in MRI	Influx of inflammatory cells into the synovial membrane, with angiogenesis, proliferation of chronic inflammatory cells	Clinical findings coupled anti-CCP antibody	Rheumatoid nodules
		Relapsing polychondritis	-	+/-	+/-	+	+	-	-	-	-	Leukocytosis, Anemia	-	Cryoglobulins, ANA, C-ANCA	Calcification of cartilaginous structures	Aortic root dilatation	Aortic root dilatation and degree of aortic regurgitation in echocardiography	Tracheal stenosis in CXR	Chondrolysis, Chondritis, Perichondritis	Clinical findings coupled with imaging	Ear pain and redness, Polyarthritis