Pulmonary nodular lymphoid hyperplasia associated with Sjögren's syndrome

Abstract

Pulmonary nodular lymphoid hyperplasia (NLH) is a term first suggested by Kradin and Mark to describe one or more pulmonary nodules or localized lung infiltrates consisting of reactive lymphoid proliferation. To date, there have been only a few cases of pulmonary NLH reported associated with autoimmune disorders. There is no case of NLH associated with Sjögren's syndrome from Korea in the medical literature. A 56-year-old woman was referred to our hospital with cough productive of sputum and chest tightness. The Computed tomography scans of the chest revealed multiple and well-defined peribronchiolar nodular opacities. A video assisted thoracoscopic surgery (VATS) biopsy was performed and the nodular opacity in the lung parenchyma was pathologically confirmed as NLH. Through meticulous review of patient's record, we found that she had been suffering from dry eye and dry mouth. The symptoms suggested Sjögren's syndrome, which was confirmed by specific laboratory tests including the Schirmer test, anti-nuclear antibody and anti-Ro/La antibody. The patient is followed regularly and has no further progression of symptoms.

Figure 1

Numerous parenchymal nodules in both upper lobe lung fields (A) and irregular thickening of the right major fissure (B).

Figure 2

Follow-up chest CT 2 months after the initial visit reveals peribronchial consolidation (A), and marked bronchial wall thickening (B) in the right middle lobe lung field.

Figure 3

¹⁸FDG PET-CT scan shows increased glucose metabolism and peribronchial consolidation in the right upper lobe (A) and middle lobe (B).

Figure 4

The Hematoxylin-eosin stain showed peribronchiolar lymphocytic infiltration and bronchiolar damage (A, ×100), fibrotic narrowing is seen in small vessels and peribronchiolar fibrosis is commonly associated with lymphocytic infiltration (B, ×200).

Figure 5

In the immunoglobulin heavy chain rearrangement analysis, a positive control with a monoclonal immunoglobulin heavy chain pattern of a lymphoma patient. The heavy chain rearrangement in our patient shows a polyclonal pattern of reactivity, different from the positive control (M: molecular marker, PC: positive control, NC: negative control, bp: base pair).