[CIS PIDD] [cis-pidd] VS: Interstitial lung disease in CVID

[CIS-PIDD]

Hi Peter,

Personally I would start and check if DLCOc improves clearly, but there is a lot of natural fluctuation in the disease and diffusion is still adequate. However, if progresses, irreversible damage often seems to come about?

What would others do?

DOSE:
Prednisolone 30-40mg is usually enough to start with in CVID, unlike in the general population (where 1mg/kg is commonly required), and a rather fast tapering to 15-20 --> 10-7.5 mg/d is usually sufficient to induce and keep the response, especially if no GLILD like manifestations. Got this rule of thumb from Helen Chapel a few years ago and has worked well for us in our few cases (whereas high dose steroids may cause septic infections in both CVIDs and e.g. AICDA patients).

Do others share this experience?

Follow diffusion, avoid excessive radiation.

(If GLILD, not just LIP, rituximab +/- MMF might be needed - see Jack's / John Routes published report and earlier discussions on Listserve by Jack, Kate Sullivan and colleagues).

ATB

Mikko



oyl Mikko Seppänen
Harvinaissairauksien yksikkö (HAKE), HUS

Mikko Seppänen, MD, PhD, Associate professor
Specialist in Internal Medicine and Infectious Diseases
Head, Rare Disease Center, Helsinki University Hospital (HUH)
Children's Hospital, P.O.Box 280
FI-00029 HUS
FINLAND
&
Senior Consultant (PIDD)
Adult Immunodeficiency Unit
Inflammation Center, HUH

phone +358 9 47180201
GSM +358 50 4279606
fax +358 9 47174703



Lähettäjä: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Lähetetty: 11. huhtikuuta 2016 14:31
Vastaanottaja: CIS-PIDD <cis-pidd at lyris.dundee.net>
Aihe: [cis-pidd] Interstitial lung disease in CVID




Dear all,



As we do not have much experience in interstitial lung disease in CVID I would like to ask you for your comment.



I am seeing  32 years old male with CVID. He presented at the age of 28 with interstitial lung disease,  surgical biopsy was performed and  lymphocyte  interstitial pneumonia was confirmed.  He also had splenomegaly, thrombocytopenia and anaemia. He was treated with SCIG,  steroids for 1 year  and rituximab for 6 months and interstitial infiltrates and haematological abnormalities resolved.



Than he was 4 years without symptoms , on IVIG replacement therapy, infections were rare and controlled. At the moment he is on subcutaneous immunoglobulin replacement.



>From September 2014 we follow slowly progression of  bilateral interstitial changes, on HRCT they are described nodular peribronchial infiltrates, with some signs of follicular bronchiolitis. The infiltrates are slowly migrating, but in progression. The radiologist suggest organizing pneumonia.

Bronchoscopy was performed two times, histology  transbronchial biopsy: nonspecific inflammatory  infiltration. Without granulomas, no sings for lymphoma or LIP or COP.

Surgical biopsy was not performed.

Bronchial lavage was positive for Pneumococci, negative for yeasts, mycobacteria. The patient underwent prolonged treatment with penicillin, without improvement.

The patient is all the time asymptomatic, with mild dyspnoea at exercise, his lung functions  is all the time normal and DLCO stable at 80%. CRP is normal.  Thrombocytes are low, but stable at around 90-100 /10*9 L, He has again slowly progression of splenomegaly.



Should we start steroid treatment for slowly progressive interstitial lung disease in CVID - despite the fact that the patient is asymptomatic, with normal lung function ?

 Thank you for your answers!



Peter Kopač



Univerzitetna klinika za pljučne bolezni in alergijo, GOLNIK
University Clinic of Respiratory and Allergic Diseases, Golnik, Slovenia

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