Medium-sized vessel vasculitis with arthralgia

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
Medium-Vessel Vasculitis	Polyarteritis nodosa		+	+	+	+	+	+	+/-	+/-	LAMP-2 protein autoantibodies	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	-	Immunofluorescent or immunoperoxidase staining of R rickettsii	Clinical criteria and tick exposure	Rash on the palms and soles
		Staphylococcal Scalded Skin Syndrome	+	+	+	+	-	-	+/-	+/-	Anti exfoliatin and anti alpha-toxin antibodies	Leukocytosis with left shift	↑	Blood culture	Pneumonia	-	-	-	Intraepidermal blister, dense superficial perivascular lymphohistiocytic infiltrate	Blood culture and clinical findings	Widespread skin erythema, fluid-filled blisters
	Systemic disease	Juvenile Idiopathic Arthritis	-	-	-	+	+/-	-	-	-	Rheumatoid factor (RF), S100A12	Lymphocytosis, Thrombocytopenia	↑	Myeloid-related proteins 8/14 (MRP8/14)	Synovial hypertrophy, Joint effusions	Cerebral vasculitis	Inflamed synovium	Bone scanning	Vascular congestion, RBC extravasation, Venular lumen occlusion	Conventional radiography	Evanescent rash, Dactylitis