[CIS PIDD] [cis-pidd] 33yo CVID, hepatopulmonary syndrome, granulomatous disease, liver transplant

[Perez, Elena]

Dear colleagues,

I would like to ask the group for feedback on a complicated case...

33yo female recently referred to me with CVID complicated by granulomatous disease involving lymph nodes, liver, spleen (s/p splenectomy several years ago), intestines, and hepatopulmonary syndrome (HPS), MELD score 25, history of two episodes of brain abscesses (resolved), on antibiotic prophylaxis with clindamycin and doxycycline, and IVIG, currently listed for liver transplant.

Patient consented to share this information.

The main question is whether to pursue rituximab/azathioprine or other immunosuppressive regimen for the granulomatous disease while awaiting liver transplant or wait on this since liver transplant is the only hope for improving the HPS. Her O2 requirement at rest is 6L NC, up from 4L. She is on the transplant list at my institution.

I presume granulomatous disease will come back eventually to the transplanted liver although maybe immunosuppressives peritransplant may ameliorate? So what is best approach?
a. Treat before transplant, maybe improve status while waiting for liver, risking delay of tx?
b.Treat after transplant to control residual granulomatous disease of ln, intestine, (lung?), in the setting of transplant related immunosuppressives?
c. Monitor / reassess after transplant for signs of granulomatous disease and start additional immunosuppressive therapy at that point?
d. other


She is high on the list for transplant and is already “on call” for the hospital. Her oxygen requirement is 6L NC with sats in 88-91% at baseline (at rest).

Liver transplant is the only hope of improving the hepatopulmonary syndrome. I will be discussing her case with transplant team, but wanted to bring some perspectives from any of you with more experience with HPS + CVID + liver tx.

Patient is a transplant nurse, and is aware of the risks, benefits, and implications.

I appreciate your comments/suggestions.
Thank you,
Elena

additional history
PMH:
--13yo diagnosed with ITP, then hosp 5d IVIG, incr plts, BM not diagnostic.
-- seen by Dr. Good at St. Pete: dx'd with CVID, chronic OM as child, pneumonia (not life threatening).
--15yo, started maintenance IVIG, receiving IVIG since then monthly (prior hematology was following and giving it q6m).
--repeat ITP episode with 30k plts in 2006 and enlarged spleen.
--spleen continued enlarged, until she became symptomatic-->splenectomy at 26y.
-- CT scan prior to splenectomy, showed a recanulized umbilical vein, indicating portal HTN.
--hepatologist discovered granulomatous disease in her liver and elevated portal pressure on transjugular biopsy. They talked about splenorenal shunt, but pressure was not so high. Didn't do anything further at that point.
--developed a diaphragmatic hernia (2007) 5m after splenectomy. It was fixed via same incision.
--enlarged LN in mediastinum worried for CA, lymphoma but it was also granulomatous disease on biopsy.
--did well for a while, and incidentally, had some esophageal varices banded prior to splenectomy.
--became worried for anemia. Her HCT lowered to 17 without explanation, there was no frank bleeding, possibility of hemolysis from IVIG, vs. Ulcer. There has been no recurrence.
--history of receiving Remicaid for GI symptoms of chronic diarrhea for 5 y. She was scoped multiple times, showing granulomatous disease in the intestines (seemed like colitis). Used remicaid for 7m, no difference, diarrhea had already subsided.
--developed fevers, for 2months constant. No source found. She was in and out of hospital. LFTs went up to 1000, admitted several times. Treated with steroids, resolved. Did well again, until 2011.
--shortness of breath, asthma, saw pulmonologist, heart was overworking, metoprolol then got better cardiac output.
--Gall stone pancreatitis in 2011. ERCP and sphincterotomy, became septic, had gall bladder removed.
--6weeks later had brain abscess, requiring craniotomy twice, same bug b-hemolytic strep from gall bladder.
-- care team realized she has hepatopulmonary syndrome.
--went to pulmonologist at Cleveland Clinic, HPS. Started O2.
--2013 January, brain infection. Had had teeth cleaned. IV antibiotics for 6m.
--had taken out port, no growth.
-- Bx negative for cirrhosis. Imaging did say cirrhosis, but probably granulomatous disease b/c liver looks nodular.
--denied tx at Mayo
--No granulomatous disease in lung known
--Antibiotics prophy: clindamycin 300mg daily, doxycyline 100mg once a day. Since brain abscess and PICC out.
--Allergies: sulfa, pcn, levaquin, Rocephin all react with rash (hive). She has taken them fine and then next time might have reaction. Desensitized to rocephin and tolerated ok. PCN is a problem, rash by time home she had reaction despite desensitization. Vanco and mero were fine.


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