Granulomatous lymphocytic interstitial lung disease

Case contributed by Tom Elswood
Diagnosis certain

Presentation

Known panhypogammaglobulinaemia (i.e. chronic immunodeficiency). Recurrent chest infections. Now presents with shortness of breath, recurrent cough, sinusitis and an abnormal CXR.

Patient Data

Age: 30 years
Gender: Female

Bilateral hilar lymphadenopathy. Air bronchograms adjacent to an ill-defined right heart border implying right middle lobe consolidation/atelectasis. Ill-defined soft tissue nodules throughout both lungs.

Diffuse central and peripheral soft tissue nodules with surrounding ground-glass change, some of which exhibit cavitation. Minor basal cylindrical bronchiectasis.

Large volume right axillary, paratracheal, subcarinal and bilateral hilar lymphadenopathy.

Partial right middle lobar collapse/consolidation.

Splenomegaly. Otherwise unremarkable upper abdominal organs.

Case Discussion

This young woman has had a diagnosis of panhypogammaglobulinaemia for years. Recently, her hematological diagnoses have been refined to common variable immunodeficiency and complete IgA deficiency. 

She now has progressive widespread lymphadenopathy and splenomegaly - awaiting lymph node biopsy. Minor thrombocytopenia only on peripheral blood film.

A recurrent issue is lower respiratory tract infections. She was well from a lung point of view at the point of this CT examination. 

From a radiology perspective, this diagnosis fits primarily due to her primary immunodeficiency. Her CT findings are relatively nonspecific and a differential in any other patient would include multifocal cavitating infection, septic emboli, rheumatoid lung disease and tuberculosis. The latter two have been excluded by alveolar lavage and rheumatological serology.

Incidentally, bronchioalveolar lavage was positive for Aspergillus antigen. However, her clinical symptoms do not fit with an invasive fungal picture.

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