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Table 1 Clinical evolution of CAPS in the described patient

From: The complex treatment including rituximab in the Management of Catastrophic Antiphospholid Syndrome with renal involvement

Organ manifestation

Clinical signs

Laboratory abnormalities typical for CAPS

Laboratory tests which were normal

Kidneys

 renal failure

Peripheral edema

Thrombocytopenia

Fibrinogen

 renal vein thrombosis

Fluid in both pleural cavities

Elongate APTT

ANA

 hematuria

 

Elevated D-dimer

Anti-dsDNA

 proteinuria

 

Reduced complement components C3 and C4

ADAMTS 13 and its inhibitor

Brain

 stroke

 

Positive lupus anticoagulant

 

Skin

 livedo reticularis

livedo reticularis

Antiβ2glicoprotein-I antibodies

 

Peripheral vessel

 peripheral venous thrombosis

Edema of the legs

Thrombotic microangiopathy in renal biopsy

 

Eye

 central retinal artery thrombosis

Blurred vision

  
  1. APTT activated partial thromboplastin time, ANA antinuclear antibodies, anti-dsDNA anti-double strength DNA antibodies, CAPS catastrophic antiphospholipid syndrome