Presentation
Known cystic lung disease presented for progress imaging
Patient Data
Multiple bilateral lung nodules. No calcification.
Multiple scattered thin-walled cysts with perivascular distribution. Vessels traverse some of the cysts.
Incidental goiter.
Case Discussion
In this patient, there was previous history of hepatitis B infection, but no current infection. The patient had a background history of Sjögren syndrome, rheumatoid arthritis, and hypothyroidism due to Hashimoto disease.
Serological testing was positive for multiple markers of disease:
IgG
serum protein electrophoresis
free light chains: free kappa and free lambda
antinuclear antibody
rheumatoid factor
extractable nuclear antigen antibodies: SSA (Ro-60), SSA (Ro-52), SSB
Pulmonary function testing showed borderline mild restrictive lung disease with no bronchodilator reversibility. The patient tested negative for HIV.
Lymphoid interstitial pneumonia (LIP) is associated with centrilobular nodular or geographic opacity, usually ground glass, sometimes consolidative. In this case, the nodules are likely to be due to LIP.
Cystic lung disease in Sjogren syndrome is commonly attributed to LIP however it is more likely to be a consequence of protein deposition: morphologically similar lung cysts are a feature of other diseases in which plasma cells produce excess immunoglobulin-associated light chains such as light chain deposition disease and monoclonal lymphoplasmacytic proliferative disorders. Macrophages are attracted to sites of protein deposition and release metalloproteinases which destroy lung tissue. This may account for the residual strands of intracystic tissues as well as the thin-walled bronchiectasis which may also be seen in Sjogren syndrome 1,2.
Co-author Liz Silverstone, with thanks to Jeffrey Kanne.
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