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Small-sized vessel vasculitis with hypertension

Introduction

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[1] + +/- – – – – + – 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[2] +/- +/- + + – – + +/- HCV RNA, Cryoglobulins Leukocytosis,Β 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[3] +/- – – – – – + – 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[4] – – – + – – + +/- 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[5] + + – + – – + + C5b-9, ADAMTS13 Anemia, Thrombocytopenia, ReticulocytosisΒ  ↑ ↑Lactate dehydrogenase (LDH), HypercalcemiaΒ  Thalami, brainstem, or cerebellum abnormality Cerebral microangiopathy or hypertension Hypoechoic kidneyΒ  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, ProteinuriaΒ 
Multiple Myeloma[6] + – + + + + +/- +/- 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[7] + + + + +/- +/- +/- +/- MYC,Β BCL2,Β BCL6, andΒ TP53 Lymphocytosis, Anemia, Thrombocytopenia ↑ ↑Lactate dehydrogenase (LDH), HypercalcemiaΒ  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[8] +/- + – +/- – – + + 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 Β Acral cyanosis, Hemorrhagic skin infarctions
Systemic disease Systemic lupus erythematosus[9] + + + + – – + + Anti dsDNA, ANA Β 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

References

  1. ↑ McAdoo SP, Pusey CD (July 2017). “Anti-Glomerular Basement Membrane Disease”. Clin J Am Soc Nephrol. 12 (7): 1162–1172. doi:10.2215/CJN.01380217. PMIDΒ 28515156.
  2. ↑ Guo QY, Wu M, Wang YW, Sun GD (2017). “Hepatitis C virus-associated cryoglobulinemia with membrano-proliferative glomerulonephritis treated with prednisolone and interferon: A case report”. Exp Ther Med. 14 (2): 1395–1398. doi:10.3892/etm.2017.4671. PMCΒ 5525644. PMIDΒ 28810602.
  3. ↑ Parambil JG, Savci CD, Tazelaar HD, Ryu JH (April 2005). “Causes and presenting features of pulmonary infarctions in 43 cases identified by surgical lung biopsy”. Chest. 127 (4): 1178–83. doi:10.1378/chest.127.4.1178. PMIDΒ 15821192.
  4. ↑ VanDeVoorde RG (January 2015). “Acute poststreptococcal glomerulonephritis: the most common acute glomerulonephritis”. Pediatr Rev. 36 (1): 3–12, quiz 13. doi:10.1542/pir.36-1-3. PMIDΒ 25554106.
  5. ↑ Corrigan JJ, Boineau FG (November 2001). “Hemolytic-uremic syndrome”. Pediatr Rev. 22 (11): 365–9. PMIDΒ 11691946.
  6. ↑ Michels TC, Petersen KE (March 2017). “Multiple Myeloma: Diagnosis and Treatment”. Am Fam Physician. 95 (6): 373–383. PMIDΒ 28318212.
  7. ↑ Shankland KR, Armitage JO, Hancock BW (September 2012). “Non-Hodgkin lymphoma”. Lancet. 380 (9844): 848–57. doi:10.1016/S0140-6736(12)60605-9. PMIDΒ 22835603.
  8. ↑ Venugopal A (2014). “Disseminated intravascular coagulation”. Indian J Anaesth. 58 (5): 603–8. doi:10.4103/0019-5049.144666. PMCΒ 4260307. PMIDΒ 25535423.
  9. ↑ Tsokos, George C. (2011). “Systemic Lupus Erythematosus”. New England Journal of Medicine. 365 (22): 2110–2121. doi:10.1056/NEJMra1100359. ISSNΒ 0028-4793.

Editor-In-Chief:
C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Eiman Ghaffarpasand, M.D. [2]