Differentiating vasculitis from other diseases

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Vasculitis

Overview

Classification

Large-sized vessel vasculitis
Takayasu's Arteritis
Temporal Arteritis
Medium-sized vessel vasculitis
Kawasaki's Disease
Polyarteritis Nodosa
Small-sized vessel vasculitis
Churg-Strauss Syndrome
Cutaneous leukocytoclastic vasculitis
Essential cryoglobulinemic vasculitis
Henoch-Schonlein Purpura
Microscopic polyangiitis
Wegener's Granulomatosis
Variable-sized vessel vasculitis
Sjogren syndrome
Cogan syndrome
Single organ vasculitis
Primary central nervous system angiitis

Causes

Differential Diagnosis

gsEditor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief:

Overview

Differential Diagnosis

Abbreviations: ABG= Arterial blood gas, ANP= Atrial natriuretic peptide, 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.

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 Other
Large-Vessel Vasculitis Takayasu arteritis[1] + +/- + - + + +/- +/- ESR Leukocytosis, Anemia ↑ ↑CRP Aneurysmal dilatation of the aorta Blood vessel stenosis Circumferential thickening of the arterial wall (Macaroni sign) PET-scan, Cardiac CT Granulomatous inflammation of arteries Arteriography Coronary aneurysm
Giant cell arteritis[2] + - + +/- - - - +/- ESR Normal ↑ ↑CRP Stenosis, Occlusion, Dilatation Aneurysm - Mural inflammation in MRA Granulomatous inflammation of arteries Biopsy  Jaw pain and claudication
Neurological disease Cerebral aneurysm[3] + - - - - - +/- + - Normal Normal - Well-defined round, slightly hyperattenuating lesion Bulging out of the main lumen - Heterogeneous signal intensity in MRA Layers of smooth muscle cells, Intact endothelium Digital subtraction angiography (DSA) Nausea, Vomiting
Neurofibromatosis type 1[4] +/- - +/- - - - + + NF1 mutated gene Normal Normal Neurofibromin gene Bone abnormalities  Optical coherence tomography angiography (OCTA) - Optic nerve gliomas in MRI Elongated spindle-shaped cells in neurofibromas NIH diagnostic criteria Cafe au Lait spot
Neurofibromatosis type 2[5] +/- - +/- - +/- - - + NF2 mutated gene Normal Normal Schwannomin Meningioma, Schwannoma, Ependymoma Fluorescein angiography showed retinal hamartoma Localized schwannomas in nerve ultrasound Schwannoma in MRI Encapsulated biphasic nerve sheath tumor NIH diagnostic criteria Hearing loss, Vision loss
Systemic disease Fibromuscular dysplasia[6] + - +/- + + + + +/- 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)
Ehlers-Danlos syndrome[7] - - - + +/- +/- - +/- TGF-β Normal Normal Cultured skin fibroblasts Multiple vascular segments with aneurysms and dissections Dissection of the posterolateral branch of the left circumflex coronary artery (LCx) Visceral arteries abnormality Vascular abnormalities in MRA Thin and rare collagen bundles in the dermis History and physical examination Bleeding, Bruisability, Heart murmur
Polymyalgia rheumatica (PMR)[8] + - +/- + - - - - Plasma fibrinogen Normocytic, normochromic anemia ↑ CRP Periodontoid localization of calcification Vessel wall thickening, Increased mural contrast enhancement Subacromial or subdeltoid bursitis High F-FDG accumulation around the joints in FDG PET-CT Small angular fibers, Pyknotic nuclear clumps, or target-targetoid fibers - Joint stiffness, Fatigue
Amyloidosis[9] +/- - - +/- +/- - + + Immunoglobulin light chain(Amyloid) Anemia Normal ↑ Cr or BUN,

↑ ALT or AST

Diffusely hypoattenuating and enlarged liver Amyloid deposition in the media and adventitia of small arteries  Solid organs increased echogenicity Tc-DPD for cardiac amyloid deposits Extracellular deposition of fibrillar proteins Biopsy Cardiomegaly, Dyspnea
Diseases Headache Fever Weight loss Arthralgia Claudication Bruit HTN Focal neurological disorder Biomarker CBC ESR Other CT scan Angiography Ultrasound Other Histopathology Gold standard Additional findings
Medium-Vessel Vasculitis Polyarteritis nodosa[10] + + + + + + +/- +/- ESR, CRP 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[11] +/- +/- + + +/- +/- + - 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[12] - + +/- + + - +/- - 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[13] + + + + - - - +/- B19 DNA, ↓Reticulocyte count Anemia ↑ anti–parvovirus B19 IgM - - Hydrops in fetal ultrasonography - - B19 DNA Purpuric rash, Erythema multiforme
Scarlet fever[14] + + +/- + - - - - 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[15] + + + + - - - +/- 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[16] + + + + - - - - 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[17] + + + + +/- - - - 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[18] +/- + +/- + +/- - - - 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[19] +/- + +/- + - - - - 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 Clinical criteria and tick exposure
Staphylococcal Scalded Skin Syndrome + + + + - - +/- +/-
Toxic Epidermal Necrolysis - + + - - - - +/-
Cardiovascular disease Atrial Myxoma - - +/- - - - - +/-
Cholesterol Embolism +/- +/- - - + - - +
Segmental arterial mediolysis + - - - + + + +/-
Systemic disease Antiphospholipid Syndrome + + - - - - - +/-
Juvenile Idiopathic Arthritis - - - + +/- - - -
Diseases Headache Fever Weight loss Arthralgia Claudication Bruit HTN Focal neurological disorder Biomarker CBC ESR Other CT scan Angiography Ultrasound Other Histopathology Gold standard Additional findings
Small-Vessel Vasculitis ANCA-associated vasculitis Microscopic polyangiitis
Granulomatosis with polyangiitis (Wegener’s)
Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
Hydralazine-associated ANCA-associated vasculitis
Immune complex small-vessel vasculitis Anti-glomerular basement membrane disease
Cryoglobulinemic vasculitis
Hepatitis C virus-associated cryoglobulinemic vasculitis +/- +/- + + - - + +/- ↓serum C4, Positive RF Palpable purpura, Microscopic hematuria
IgA vasculitis (Henoch-Schönlein purpura)
Hypocomplementemic urticarial vasculitis (anti-C1q vasculitis)
Gastrointestinal disease Acute mesenteric ischemia
Cardiovascular disease Infective Endocarditis
Leukocytoclastic Vasculitis
Pulmonary disease Langerhans Cell Histiocytosis
Lung Abscess
Non-Small Cell Lung Cancer
Small Cell Lung Cancer
Asthma
Pulmonary Infarction
Renal disease Acute Poststreptococcal Glomerulonephritis
Hematologic disease Hemolytic-Uremic Syndrome
Chronic Lymphocytic Leukemia (CLL)
Multiple Myeloma
Hypereosinophilic Syndrome
Non-Hodgkin Lymphoma
Serum Sickness
Disseminated Intravascular Coagulation
Idiopathic Thrombocytopenic Purpura
B cell lymphoproliferative disorder
Systemic disease Sarcoidosis
Legionella Infection
Systemic lupus erythematosus
Rheumatoid arthritis
Relapsing polychondritis
Diseases Headache Fever Weight loss Arthralgia Claudication Bruit HTN Focal neurological disorder Biomarker CBC ESR Other CT scan Angiography Ultrasound Other Histopathology Gold standard Additional findings
Variable-vessel vasculitis Behçet’s syndrome
Cogan's syndrome
Gastrointestinal disease Inflammatory Bowel Disease
Whipple's disease
Sjögren's syndrome
Single-organ vasculitis Primary central nervous system vasculitis
Infectious disease Aspergillosis
Histoplasmosis
Herpes Simplex Encephalitis
Systemic disease Eclampsia
Fibromuscular Dysplasia

References

  1. ↑ Vaideeswar P, Deshpande JR (2013). "Pathology of Takayasu arteritis: A brief review". Ann Pediatr Cardiol. 6 (1): 52–8. doi:10.4103/0974-2069.107235. PMC 3634248. PMID 23626437.
  2. ↑ Calvo-Romero JM (2003). "Giant cell arteritis". Postgrad Med J. 79 (935): 511–5. PMC 1742823. PMID 13679546.
  3. ↑ Stafa A, Leonardi M (2008). "Role of neuroradiology in evaluating cerebral aneurysms". Interv Neuroradiol. 14 Suppl 1: 23–37. doi:10.1177/15910199080140S106. PMC 3328052. PMID 20557771.
  4. ↑ Cassiman C, Casteels I, Stalmans P, Legius E, Jacob J (2017). "Optical Coherence Tomography Angiography of Retinal Microvascular Changes Overlying Choroidal Nodules in Neurofibromatosis Type 1". Case Rep Ophthalmol. 8 (1): 214–220. doi:10.1159/000469702. PMC 5422752. PMID 28512424.
  5. ↑ Evans, D G. R (2000). "Neurofibromatosis type 2". Journal of Medical Genetics. 37 (12): 897–904. doi:10.1136/jmg.37.12.897. ISSN 1468-6244.
  6. ↑ Plouin PF, Perdu J, La Batide-Alanore A, Boutouyrie P, Gimenez-Roqueplo AP, Jeunemaitre X (2007). "Fibromuscular dysplasia". Orphanet J Rare Dis. 2: 28. doi:10.1186/1750-1172-2-28. PMC 1899482. PMID 17555581.
  7. ↑ Gazit Y, Jacob G, Grahame R (2016). "Ehlers-Danlos Syndrome-Hypermobility Type: A Much Neglected Multisystemic Disorder". Rambam Maimonides Med J. 7 (4). doi:10.5041/RMMJ.10261. PMC 5101008. PMID 27824552.
  8. ↑ Michet CJ, Matteson EL (2008). "Polymyalgia rheumatica". BMJ. 336 (7647): 765–9. doi:10.1136/bmj.39514.653588.80. PMC 2287267. PMID 18390527.
  9. ↑ Baker KR, Rice L (2012). "The amyloidoses: clinical features, diagnosis and treatment". Methodist Debakey Cardiovasc J. 8 (3): 3–7. PMC 3487569. PMID 23227278.
  10. ↑ Howard T, Ahmad K, Swanson JA, Misra S (2014). "Polyarteritis nodosa". Tech Vasc Interv Radiol. 17 (4): 247–51. doi:10.1053/j.tvir.2014.11.005. PMC 4363102. PMID 25770638.
  11. ↑ Sharma A, Sharma K (September 2013). "Hepatotropic viral infection associated systemic vasculitides-hepatitis B virus associated polyarteritis nodosa and hepatitis C virus associated cryoglobulinemic vasculitis". J Clin Exp Hepatol. 3 (3): 204–12. doi:10.1016/j.jceh.2013.06.001. PMC 4216827. PMID 25755502.
  12. ↑ Takahashi K, Oharaseki T, Yokouchi Y (2011). "Pathogenesis of Kawasaki disease". Clin Exp Immunol. 164 Suppl 1: 20–2. doi:10.1111/j.1365-2249.2011.04361.x. PMC 3095860. PMID 21447126.
  13. ↑ Heegaard ED, Brown KE (2002). "Human parvovirus B19". Clin Microbiol Rev. 15 (3): 485–505. PMC 118081. PMID 12097253.
  14. ↑ Basetti S, Hodgson J, Rawson TM, Majeed A (2017). "Scarlet fever: a guide for general practitioners". London J Prim Care (Abingdon). 9 (5): 77–79. doi:10.1080/17571472.2017.1365677. PMC 5649319. PMID 29081840.
  15. ↑ Vostral SL (2011). "Rely and Toxic Shock Syndrome: a technological health crisis". Yale J Biol Med. 84 (4): 447–59. PMC 3238331. PMID 22180682.
  16. ↑ Balfour HH, Dunmire SK, Hogquist KA (2015). "Infectious mononucleosis". Clin Transl Immunology. 4 (2): e33. doi:10.1038/cti.2015.1. PMC 4346501. PMID 25774295.
  17. ↑ Levett PN (April 2001). "Leptospirosis". Clin. Microbiol. Rev. 14 (2): 296–326. doi:10.1128/CMR.14.2.296-326.2001. PMC 88975. PMID 11292640.
  18. ↑ Biesiada G, Czepiel J, Leśniak MR, Garlicki A, Mach T (2012). "Lyme disease: review". Arch Med Sci. 8 (6): 978–82. doi:10.5114/aoms.2012.30948. PMC 3542482. PMID 23319969.
  19. ↑ White SJ, Boldt KL, Holditch SJ, Poland GA, Jacobson RM (2012). "Measles, mumps, and rubella". Clin Obstet Gynecol. 55 (2): 550–9. doi:10.1097/GRF.0b013e31824df256. PMC 3334858. PMID 22510638.

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