11 Apr 2021

1/14/21: AIDS Cholangiopathy

PGY2 Allison Wang presented a case of a 57 year old male with history of AIDS (CD4 62 9/2020), HFrEF with EF 47% on TTE 11/2019 and CKD3 who presents with 3 weeks of progressive abdominal distention and 1 week of SOB.  He denies any changes in urination and has not been on a diuretic since 12/2019 (hospitalized for pre-renal AKI and hyperkalemia).  He has never had a paracentesis before.

ROS: + weight gain, +icterus, +edema, +jaundice, no rashes

Ancillary history only significant for medications (abacavir, dolutegravir-rilpivirine, lisinopril, carvediolol, atovaquone and cholecalciferol).  He is born in Mexico and immigrated to US in 1980s.  Used to drink 2-3 beers/week but has been sober x5 years and smokes 4 cigarettes/day for last 14 years.  Former meth user, denies any IVDU.

Vitals: T 36.9, HR 98, RR 18, BP 128/72, O2 sat 94% on 3L NC

Physical Exam significant for cachectic Spanish speaking male, +scleral icterus, normal cardiac exam, tachypneic, shallow breaths on 3LNC, tense and distended abdomen with shifting dullness, rectal negative for blood/melena, 3+ b/l LE edema to thigh, +spider angiomas and +jaundice.

CBC: WBC 2.2> Hb 6.5/Hct 19.5 <Plt 32 (MCV 98.6, RDW 17.5%)

CMP:

Na 131/K 4.6/Cl 100/HCO3 15/BUN 43/Cr 3.06<Glucose 110 (Baseline Cr: 2)

ALP 234>T.protein 6.3/Albumin 1.3/AST 28/ALT 14/Tbili 5.4/Dbili 5

Ca 8.7, Mg 1.7, Phos 2.3

Coags: PT 23.3, INR 2.09, PTT 51.0

Infectious workup initiated, with the following findings:

  • BCx: 4/4 pansensitive E. coli
  • Lactate elevated 7.5
  • CD4: 62
  • EBV IgG elevated, negative IgM
  • CMV IgG elevated, negative IgM
  • Histoplasmosis negative, Aspergillus negative, Toxoplasmosis negative
  • RPR negative
  • GC/CT negative
  • Coxiella negative
  • AFB sputum negative x3, MTB PCR negative x2

Heme workup:

  • Iron Panel significant for anemia of chronic disease
  • Fibrinogen: 157
  • DDimer: 3.90
  • Factor 8 assay: 228%

Renal workup:

  • UA with trace protein, small bilirubin, large blood (26-50 RBC), large leukocyte (11-30 WBC), moderate bacteria
  • Uprotein/cr: 0.61
  • FeNa: 0.5%
  • FeUrea 9.3%

Hepatology workup:

  • Utox negative, ETOH negative
  • Hep A, B, C, E negative
  • Paracentesis analysis
    • Nucleated cell: 189/cumm (64% PMNs)
    • Albumin BF: 0.6 g/dL (serum albumin 2.8)
    • Protein BF: 1.7 g/dL
    • Culture: no growth x 5days, no AFB isolates at 7 weeks
  • Bile Acids: Cholic Acid (45.1), Chenodeo Acid (41.1), Total bile acids (86.2)
  • PFIC mutation is negative
  • ACE: 35
  • ANA, ANCA negative
  • Anti-actin, anti-LKM, anti-mitochondrial M2 all negative
  • IgA, IgM, IgG all elevated

Abd U/S showed nodular hepatic surface, splenomegaly and moderate to large amount of abdominal ascites are seen, suggestive of portal hypertension.  Gallbladder wall thickening with cholelithiasis.

Trans-jugular liver biopsy was performed which showed the following pressures:

  • R atrium pressure: 10mmHg
  • Free hepatic vein pressure: 16-19mmHg
  • Wedge pressure: 21-23 mmHg
  • Portal systemic gradient: 10-14 mmHg
  • Pathology: portal fibrosis with focal early bridging, prominent portal lymphocytic infiltrates with increased plasma cells, moderate periportal interface inflammatory reaction, focal interlobular bile duct loss, portal and lobular granulomatous inflammation, scant to 1+ steatosis and cholestasis.

Treatment: Patient’s acute issues were addressed included septic shock, EGD with EVL x1 done for hematemesis and CRRT for ATN.  Goals of care discussion done and patient elected to be DNR/DNI/no HD and he was discharged to SNF.

Teaching points:

  1. AIDS Cholangiopathy = Chronic infection leading to biliary strictures which cause biliary obstruction leading to cholestatic liver damage, liver failure and cholangiocarcinoma; usually occurs in patients with CD4 <100
  2. Diagnosis: Diagnosis of exclusion
    • Labs will show cholestatic liver labs, biliary strictures on imaging, and patients can get liver biopsies to look for supportive (but not diagnostic) evidence of AIDS Cholangiopathy
  3. Treatment: restart HAART so that patient increases their CD4 count

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