대한흉부영상의학회 Korean Society of Thoracic Radiology

Discussion

Diagnosis With Brief Discussion

Diagnosis

Lymphocytic interstitial pneumonia and malignant lymphoma with Sjogren disease

Radiologic Findings

Initial chest radiography shows increased peripheral predominant opacities, poorly defined nodules and suspicious reticulation in both lungs. Mild right pleural thickening is also seen (figure 1).
On initial thin-section chest CT scans, relatively diffuse ground-glass opacities and poorly defined centrilobular micronodules are noted in both lungs (figure 2, 3). There is also reticulation with thickening of the intralobular lines and interlobular septae associated with mild traction bronchiolectasis in the subpleural areas of both basal lungs.
Four years later, follow-up chest radiography shows the progression of diffuse interstitial thickening with reticular opacities in both lungs. There are newly appeared airspace consolidations in the left lower lung, as well as mild mediastinal widening with increased opacity and left pleural effusion (figure 4). Chest CT lung window scan shows further progress and increased coarseness of reticulation in the subpleural areas of both basal lungs (figure 5, 6). There are newly appeared airspace consolidations as well as large nodules and diffuse thickening of the bronchovascular bundles and interlobular septae in the left lung. Conglomerated lymphadenopathy is newly noted at the left supraclavicular (figure 7), as well as the mediastinal and left axillary (figure 8) areas. A small amount of pleural effusion is also seen in the left lung.
An open lung biopsy was performed at the initial presentation and the case was histopathologically diagnosed as lymphocytic interstitial pneumonia (LIP).
Four years later, malignant lymphoma (T-cell/histiocyte rich large B-cell type) was diagnosed using a bone marrow biopsy and excision biopsy of the left axillary lymph nodes.

Brief Review

Lymphocytic interstitial pneumonia (LIP) is a benign lymphoproliferative disorder characterized by diffuse proliferation of polyclonal lymphocytes and plasma cells in the pulmonary parenchymal interstitium, with lymphoid follicles and germinal centers. It may be associated with a variety of autoimmune and lymphoproliferative disorders including Sjögren’s disease, myasthenia gravis, systemic lupus erythematosus, multicentric Castleman’s disease and HIV infection. LIP is substantially more common in women, and most commonly presents in the fourth to seventh decades. Dyspnea and a nonproductive cough are common and may be present for months to years. PFTs often show restrictive patterns depending on the progression of the disease.

Sjögren’s disease is characterized by both dryness in the mouth and eyes as well as lymphocytic infiltration of the salivary glands, which are easily accessible and thus facilitate diagnosis. The disease can extend beyond the exocrine glands, resulting in systemic manifestations such as vasculitis, lung, renal or neurological involvement. Sjögren’s disease is also known to increase the risk of lymphoma in patients.

LIP occurring specifically in Sjögren’s disease patients is characterized by ground-glass abnormalities due to homogenous lymphocytic infiltration. Since LIP is a hyperplastic disorder of the mucosa-associated lymphoid tissues, it lies on a disease spectrum from follicular bronchiolitis to lymphoma. CT findings of LIP include ground-glass opacity, poorly defined centrilobular nodules, bronchovascular thickening, and septal thickening. Discrete lung cysts measuring 5-30 mm are often present and are ascribed to bronchiolar obstruction with lymphocytic wall infiltration. Such cysts are helpful in distinguishing LIP from malignant lymphoma, which should be suspected in the case of airspace consolidations, large nodules (>1cm), or pleural effusions.

The lymphoproliferative disorders in Sjögren’s disease can develop as part of a multi-step process in which polycolonality is followed by monoclonality and t(14;18) chromosomal translocation. Previous study reported a 30% rate of progression from LIP to malignant lymphoma. This case shows a patient with Sjögren’s disease and initial presentation of LIP who eventually developed malignant lymphoma during the course of their hospitalization.

Please refer to

Case 313,

Thoracic Imaging 2003- Korean & Japanese Film Interpretation -  Case 8,

References

1. Honda O, Johkoh T, Ichikado K et-al. Differential diagnosis of lymphocytic interstitial pneumonia and malignant lymphoma on high-resolution CT. AJR Am J Roentgenol. 1999;173 (1): 71-4
2. Johkoh T, Müller NL, Pickford HA et-al. Lymphocytic interstitial pneumonia: thin-section CT findings in 22 patients. Radiology. 1999;212 (2): 567-72
3. Das S, Miller RF. Lymphocytic interstitial pneumonitis in HIV infected adults. Sex Transm Infect. 2003;79 (2): 88-9
4. Cha SI, Fessler MB, Cool CD et-al. Lymphoid interstitial pneumonia: clinical features, associations and prognosis. Eur. Respir. J. 2006;28 (2): 364-9
5. Flament T, Bigot A, Chaigne B et-al. Pulmonary manifestation of Sjogren’s syndrome. Eur Respir Rev. 2016;25(140): 110-23

Keywords

Lung, Interstitial pneumonia, Sjogren syndrome with Lymphoproliferative disease ,