Management of granulomatous lymphocytic interstitial lung disease in a patient with common variable immune deficiency

Abstract

A 61-year-old woman presented with longstanding cough and progressive dyspnoea. She underwent an extensive evaluation and was diagnosed with common variable immunodeficiency (CVID) with granulomatous lymphocytic interstitial lung disease (GLILD). She was initially treated with subcutaneous immunoglobulin therapy, having declined intravenous immunoglobulin (IVIG) therapy. She also declined treatment with oral glucocorticoids. Over several months, she became increasingly symptomatic and developed increased pulmonary infiltrates, pleural effusions, mediastinal adenopathy, splenomegaly, pancytopenia and ascites. An interdisciplinary team composed of an immunologist, pulmonologist and haematologist deliberated over a therapeutic management approach. The patient received a recently reported immunotherapy regimen with azathioprine and rituximab. The therapy led to rapid improvement of her constitutional and respiratory symptoms, with clinical and radiographic improvement in her interstitial lung disease, lymphadenopathy, pleural effusions and ascites. This case report reviews the literature surrounding the diagnosis and management of GLILD.

Figure 1

Mild lymphocytic infiltrate involving small airways and swollen alveolar septae consistent with lymphocytic bronchiolitis.

Figure 2

Inflammatory changes in the interstitium consistent with interstitial pneumonitis.

Figure 3

Presence of organising pneumonia due to granulation tissue that fills bronchiolar and alveolar lumen.

Figure 4

Organised collection of macrophages composing minute granulomata.

Figure 5

CT of the chest demonstrating bilateral pleural effusions and peribronchial thickening.

Figure 6

CT of the abdomen demonstrating splenomegaly and ascites.

Figure 7

CT of the chest demonstrating resolution of bilateral pleural effusions 3 months after initiation of rituximab and azathioprine.

Figure 8

CT of the abdomen with contrast demonstrating resolution of ascites after 3 months of therapy.

Figure 9

(A and B) Lateral views of chest X-ray images before and after 3 months of therapy.