Sjögren’s Syndrome with Multiple Bullae Caused by Lymphocytic Interstitial Pneumonia

Excerpt

The mechanism of parenchymal bulla formation is poorly understood. It has been postulated that the mechanism of bullae formation probably involves inflammatory cell infiltration into the bronchial wall, resulting in narrowing of the airways. Thus, cell infiltration acts as a check valve mechanism [1]. Although several pulmonary disorders have been described in patients with Sjögren’s syndrome, cystic or bullous lung disease is rare [2]. Because our patient was of child-bearing age and a nonsmoker, we needed to determine whether the bullae was associated with Sjögren’s syndrome or caused by lymphangioleiomyomatosis (LAM). The bullae that are characteristic of LAM are 5–15 mm in diameter, have thin walls, and are homogeneously distributed in all lung fields [3]. In the present case, however, the bullae formation was predominant in the middle and lower lung zones (Fig. 1). Furthermore, the walls of the bullae were irregular and their sizes varied from 5 to 60 mm in diameter. Consequently, the bullae in patients with Sjögren’s syndrome differ from those in patients with LAM. Although a rare occurrence, it is imperative to consider that multiple bullous formation can be associated with Sjögren’s syndrome.

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