Systemic causes of cholestasis

Abstract

Systemic causes of cholestasis constitute a diverse group of diseases across organ systems. The pathophysiology of cholestasis in systemic disease can be a consequence of direct involvement of a disease process within the liver or extrahepatic biliary system or secondary to immune-mediated changes in bile flow. Evaluating a patient with cholestasis for a systemic cause requires an understanding of the patient's risk factors, clinical setting (eg, hospitalized or immunosuppressed patient), clinical features, and pattern of laboratory abnormalities.

Fig. 1

Histopathology of hepatic syphilis, showing portal and lobular granulomas associated with mixed inflammation (hematoxylin-eosin, original magnification, ×10).

Fig. 2

Histopathology of hepatic sarcoidosis, showing portal tract with multiple noncaseating epithelioid granulomas encased in fibrosis characteristic of hepatic sarcoid (hematoxylin-eosin, original magnification, ×10).

Fig. 3

Histopathology of hepatic amyloidosis. (A) Liver biopsy specimen showing massive sinusoidal infiltration by waxy pink amyloid deposits (hematoxylin-eosin, original magnification, ×20). (B) Congo Red stain of the same biopsy specimen viewed under polarized light shows apple-green birefringence, confirming amyloid (original magnification, ×20).

Fig. 4

Algorithm for the evaluation of the patient with systemic cholestasis. ACE, angiotensin-converting enzyme level; ALP, alkaline phosphatase level; AMA, antimitochondrial antibodies; CMV, cytomegalovirus; EBV, Epstein-Barr virus; ERCP, endoscopic retrograde cholangiopancreatography; GI, gastrointestinal; GVHD, graft-versus-host disease; LDH, lactate dehydrogenase level; MAC, Mycobacterium avium complex; MRCP, magnetic resonance cholangiopancreatography; MRI, magnetic resonance imaging; PET-CT, positron emission tomography with computed tomography; PTLD, posttransplant lymphoproliferative disorder; TB, tuberculosis; US, ultrasonography.