[CIS PIDD] [cis-pidd] Evans Syndrome, iatrogenous CVID, granulomatous LIP

[Ravishankar.Sargur at sth.nhs.uk]

Dear Markus,


We have a patient with CVID + APECED with autoimmune liver disease & autoimmune interstitial lung disease ( in the LIP spectrum). She is also positive for the newly described

Anti - BPIFB1 antibodies - " "BPIFB1 Is a Lung-Specific Autoantigen Associated with Interstitial Lung Disease. Sci Transl

Med. 2013 Oct 9;5(206):206ra139. PubMed PMID:  24107778.



You could ask Anthony Shum to look for KCNRG & BPIFB1 abs.

She was on AZA for a long time for her Autoimmune hepatitis.

Lungs stated to deteriorate and we feared she is heading for a lung transplant.

We started on RTX early this year. Significant improvement in lung function.  No significant side effects.

Hope this helps.

Ravi

Dr Ravishankar Sargur
MD, FRCPath, FRCP
Consultant Clinical Immunologist & Clinical Lead
Immunology, NGH
Sheffield Teaching Hospitals Foundation NHS Trust.

Honorary Senior Lecturer
Dept of Infection and Immunity
University of Sheffield

Training Programme Director
Immunology
East Midlands Deanery and Yorkshire & Humber Deanery

Sheffield
United kingdom
0044 114 27 15704
Email : Ravishankar.sargur at sth.nhs.uk<mailto:Ravishankar.sargur at sth.nhs.uk>
________________________________
From: Markus Seidel [mailto:markus.seidel at medunigraz.at]
Sent: 06 November 2013 21:44
To: CIS-PIDD
Subject: AW: [cis-pidd] Evans Syndrome, iatrogenous CVID, granulomatous LIP

Thanks! B12 and sFasL were normal.
Yours, Markus

Von: Nacho Gonzalez [mailto:nachgonzalez at gmail.com]
Gesendet: Mittwoch, 06. November 2013 22:26
An: CIS-PIDD
Betreff: Re: [cis-pidd] Evans Syndrome, iatrogenous CVID, granulomatous LIP

I´d try to be sure ALPS has been r/o: B12, sFASL ? (despite DNT borderline)
The best approach for GLILD was recently published JCI Jan (AZP + RTX better than one alone) http://www.ncbi.nlm.nih.gov/pubmed/22930256

We have tried in one patient (AZP and RTX) and worked. In other, RTX alone improved GLILD for several years.

Hope this helps,

Luis Ignacio Gonzalez-Granado
Immunodeficiencies.
Hematology. Oncology
Hospital 12 octubre.
Madrid. Spain


2013/11/6 Markus Seidel <markus.seidel at medunigraz.at<mailto:markus.seidel at medunigraz.at>>
Dear Colleagues,
what would be your suggestions in the therapeutic dilemma in a young adult who
has been treated successfully with MMF for 5 years for Evans Syndrome, under that time had a CVID-like B-cell phenotype (<100/µL B cells, absence of csmB cells, IgG2 and IgA-def.), did not require Ig-substitution, no severe infections,
initially had severe pancytopenia, autoimmune hemolytic anemia,
who then developed granulomatous lymphocytic interstitial pneumonitis early this year (LIP, either as consequence of iatrogenous CVID or occupational dust exposure; no infectious agent identified)
and when IS was terminated, had recurrence of severe thrombocytopenia (<10.000/µL), neutropenia (600-900/µL), autoantibodies against neutrophils, platelets, pos. Coombs test, but recovered clinically from LIP, which now appears to be stable in Chest CT.
his mother suffered from granulomatosis with polyangiitis (GPA) - but no other than blood-cell tissue- or nuclear autoantibodies are detectable in the patient.

I fear that rituximab or other systemic IS would exacerbate his LIP, which has been stable for 4 months now under rapamycin (the latter did not improve his thrombocytopenia, though); the B cells recovered to >200/µL and >2% csmB cells after discontinuation of MMF, but are now lower again under rapamycin, DNT cells borderline increased 3-5% of CD3;
Romiplostim works short time (1-2 weeks), but doesn't look like a very good long term option;
Splenectomy?, Danazole?, AZT?
...SCT?

Thanks for your comments or thoughts,
Sincerely,
Markus Seidel


Markus G. Seidel, M.D., Assoc.Prof.
Consultant| Dept.of Pediatric Hematology-Oncology | Univ.Clinics of Pediatric and Adolescent Medicine | Medical University Graz | Auenbruggerpl. 34/2 | A-8036 Graz | Austria | T. 0043 316 385 80215<tel:0043%20316%20385%2080215>| F. 0043 316 385 13717<tel:0043%20316%20385%2013717> | Secr. 0043 316 385 13485<tel:0043%20316%20385%2013485> |
Coordinator of the Working Group for Pediatric Immunology of the Austrian Society of Pediatrics and Adolescent Medicine

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