Medium-sized vessel vasculitis with focal neurological sign

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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[1] + + + + + + +/- +/- 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
Infectious disease Parvovirus B19 infection[2] + + + + - - - +/- B19 DNA, ↓Reticulocyte count Anemia ↑ anti–parvovirus B19 IgM - - Hydrops in fetal ultrasonography - - B19 DNA Purpuric rash, Erythema multiforme
Toxic shock syndrome[3] + + + + - - - +/- 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
Staphylococcal Scalded Skin Syndrome[4] + + + + - - +/- +/- 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
Toxic Epidermal Necrolysis[5] - + + - - - - +/- MicroRNA-124 Normochromic normocytic anemia, Eosinophilia ↑ Fluid loss and electrolyte abnormalities Tracheobronchial inflammation - - - Necrotic keratinocytes with full-thickness epithelial necrosis Histopathology and clinical findings Erythematous macular rash with purpuric centers
Cardiovascular disease Atrial Myxoma[6] - - +/- - - - - +/- Calretinin Mild anemia, Leukocytosis ↑ ↑IL-6 Atrial filling defect larger than a thrombus - Tumor location, size, attachment, and mobility in echocardiography Size, shape, and surface characteristics in MRI Lipidic cells embedded in a vascular myxoid stroma Echocardiography Dyspnea on exertion, Syncope
Cholesterol Embolism[7] +/- +/- - - + - - + IL-5 Eosinophilia, Leukocytosis   ↑ Eosinophiluria Thoracic and abdominal aortic sources of embolism Atheroembolism in abdominal aorta and the lower extremity arteries Excluding an intracardiac source of embolism with echocardiography  - Birefringent crystals or biconvex needle-shaped ghostly clefts within the arterial lumen Angiography  Livedo reticularis,

Ischemic patches

Segmental arterial mediolysis[8] + - - - + + + +/- - Leukocytosis - - Visceral artery aneurysm in CT angiography Alternating aneurysms and stenoses (beading) Retroperitoneal hematoma - Disruption of the smooth muscle in the media Angiography  Hematuria, Ischemic colitis
Systemic disease Antiphospholipid Syndrome[9] + + - - - - - +/- Antiphospholipid antibodies Thrombocytopenia, Hemolytic anemia - Lupus anticoagulant (LA) Stroke,

Pulmonary embolism, Budd-Chiari syndrome

Thrombus in major vessels Valve thickening, vegetations, or insufficiency in echocardiography - Noninflammatory bland thrombosis without perivascular inflammation Hx of thrombosis and antiphospholipid antibodies Miscarriage, Pulmonary hypertension

References

  1. ↑ 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.
  2. ↑ Heegaard ED, Brown KE (2002). "Human parvovirus B19". Clin Microbiol Rev. 15 (3): 485–505. PMC 118081. PMID 12097253.
  3. ↑ Vostral SL (2011). "Rely and Toxic Shock Syndrome: a technological health crisis". Yale J Biol Med. 84 (4): 447–59. PMC 3238331. PMID 22180682.
  4. ↑ Mishra AK, Yadav P, Mishra A (2016). "A Systemic Review on Staphylococcal Scalded Skin Syndrome (SSSS): A Rare and Critical Disease of Neonates". Open Microbiol J. 10: 150–9. doi:10.2174/1874285801610010150. PMC 5012080. PMID 27651848.
  5. ↑ Hoetzenecker W, Mehra T, Saulite I, Glatz M, Schmid-Grendelmeier P, Guenova E; et al. (2016). "Toxic epidermal necrolysis". F1000Res. 5. doi:10.12688/f1000research.7574.1. PMC 4879934. PMID 27239294.
  6. ↑ MacGowan SW, Sidhu P, Aherne T, Luke D, Wood AE, Neligan MC, McGovern E (June 1993). "Atrial myxoma: national incidence, diagnosis and surgical management". Ir J Med Sci. 162 (6): 223–6. PMID 8407260.
  7. ↑ Avci G, Akoz T, Gul AE (2009). "Cutaneous cholesterol embolization". J Dermatol Case Rep. 3 (2): 27–9. doi:10.3315/jdcr.2009.1031. PMC 3157794. PMID 21886725.
  8. ↑ Chao, Christine (2009). "Segmental Arterial Mediolysis". Seminars in Interventional Radiology. 26 (03): 224–232. doi:10.1055/s-0029-1225666. ISSN 0739-9529.
  9. ↑ Chaturvedi S, McCrae KR (2015). "The antiphospholipid syndrome: still an enigma". Hematology Am Soc Hematol Educ Program. 2015: 53–60. doi:10.1182/asheducation-2015.1.53. PMC 4877624. PMID 26637701.

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