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

[CIS-PIDD]

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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