If the patient has ocular involvement- Dry eye syndrome- the latest apptoach is Cyclosporin eye drops - Restasis

Dear Dr Marianne,

thank you for your comment. 

But the problem here is how to treat severe organ involvement of Sjögren's syndrome associated to PBC. 

Dear Dr. Houman,

You bring up an interesting question - the two diseases have a lot of interesting "overlap" in their immunopathogenesis and have associated "epithelitis" described for their pathology.

As far as treatment is concerned, I don't think the literature has a lot of convincing data available especially when you look at both the diseases in combination. Recent trials indicate a decent response to extra glandular Sjogren's Syndrome (but not much effect on sicca symptoms). There is however only a modest to moderate response reported in PBC patients, with moderate relief seen in patients who have failed treatment with ursodeoxycholic acid. Data on other anti-CD20 therapies is very limited.

So even though I do not have a patient yet with both, I think a trial of Rituximab in patients with "severe" (I assume you mean extra glandular symptoms) Sjogren's AND PBC that has failed initial trial of conservative ursodeoxycholic acid therapy, my next option would be a trial of rituximab.

Thanks for sharing your question!

Gaurav

mycophenolate can be given as a maintenance agent after rituximab- role of the latter in PBC is not very clear

Dear Colleague

It is important better define the clinical profile of your Sjogren syndrome. Treatment of PNS involvement may be different from that of lung involvement. On the other hand are you sure that no additional CTDs are in overlap with this Ss? If anti Ro Ab were + did you exclude an antisynthetase syndrome? Are jo-1 pl-7,etc Ab negative? These Ab frequently co occur with anti Ro ab. 

Dear Dr Cavagna 

thank you for your comments.

no other CTD is in overlap in this case. 

Anti Jo1 antibodies were negative. I exclude an antisynthetase syndrome. 

Ok I think that also othrr antisynthetase soecificities are negative If the problem is PNS;  high dose I.V. Ig for 5 days, preceeded or not by corticosteroid pulses.  If ILD: lymphocitic alveolitis high dose corticostetoids. If not....check for duagnosis. These are EBM and referenced possibilities. Rituximab is effective and also belimumab but the spectrum of possibilitirs is too wide to discuss with available onformation. Just one suggestion: did you check for concomitant mixed cryoglobulinemia?

Oh yes , cryoglobulinemia is also negative.

thank you for your pertinent suggestions. 

Put the patient on a Palaeo Diet. Use this one.

http://www.ibsexplained.com/the_easy_palaeo_diet.pdf

I can see interesting discussion about primary Sjogrens syndrome and "overlapping" or coexisting ? diseases as PBC. I think - to be onest,  that we do not a have good solution and long experience. The question is : which severe symptoms dominate pSS or PBC. We have currently treatment for pSS as GKS, Azathiopryne(for consyderation- also in coexisting autoimmune hepatitis),  CF, and in sever cases with organ involvement and autoantibodies production and high risk of lymphoma the RTX probably could be good option., plasmaphersis IVIG(discuss?). Often PBc in picture of pSS is quite mild.

In severe cases of PBC the liver transplantation may be considered

I know that it is not a clear solution but voice in disscussion.

Best regards

Maria

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Dear Dr. Houman,

It is an interesting question .

Since your patient has Sjögren´s disease and PBC, I wonder  for the posibility of IgG4 related diseases. In this case rituximab would be the choice

Best regrads

Dear Dr Rosas,

thank you for your very pertinent idea.

eventhough, in our patient we didn't observed, lymphadenopathy, pancreatitis, tubbulointertitial nephritis, nor retropetoneal fibrosis, and that there was no storiform pattern in the histology of major salivary glands biopsy, and also that PBC ARE VERY RARELY REPORTED in IG4 related disease...we will look for Ig4 elevation in our case.

thank you again. 

Thanks to a very good question but the answer dr.Jose Rosas and the case which are rare in practice, but it has deteriorated and are complicated to treat because the drugs used for SS are contraindicated for PBC and I think that suggestion is a very good recommendation for Rituximab in treatment of these very complicated and complex cases!