Chronic Epstein-Barr virus infection causing both benign and malignant lymphoproliferative disorders / 소아과

The Epstein-Barr virus (EBV) is oncogenic and can transform B cells from a benign to a malignant phenotype. EBV infection is also associated with lymphoid interstitial pneumonia (LIP). Here, we report the case of a 14-year-old boy who was diagnosed with a latent EBV infection and underlying LIP, without any associated immunodeficiency. He had been EBV-seropositive for 8 years. The first clinical presentations were chronic respiratory symptoms and recurrent pneumonia. The symptoms worsened in the following 2 years. The results of in situ hybridization were positive for EBV, which led to a diagnosis of LIP. The diagnosis was confirmed by the results of a thoracoscopic lung biopsy. The EBV titer of the bronchoalveolar lavage specimens obtained after acyclovir treatment was found to be fluctuating. The patient had latent EBV infection for 8 years, until presented at the hospital with intermittent abdominal pain and distension. Physical examination and pelvic computed tomography revealed a large mesenteric mass. A biopsy of the excised mass led to a diagnosis of Burkitt's lymphoma (BL). The patient received combination chemotherapy for 4 months, consisting of vincristine, methotrexate, cyclophosphamide, doxorubicin, and prednisolone. He is now tumor-free, with the LIP under control, and is being followed-up at the outpatient clinic. This is the first report of a Korean case of chronic latent EBV infection that developed into LIP and BL in a nonimmunocompromised child.

Lymphoid Interstitial Pneumonia Associated with Primary Sjogren's Syndrome: A Case Report / 결핵및호흡기질환

Lymphoid interstitial pneumonia (LIP) is a rare benign lymphoproliferative interstitial lung disease. LIP has been associated with autoimmune disorders, HIV, viral infections, and so on. Once underlying systemic diseases have been excluded, a diagnosis of idiopathic LIP can be made. Although 6 cases of pathologically confirmed LIP have occurred in Korea, thus far none has been associated with primary Sjogren's syndrome. A 44-year-old man was admitted to hospital due to a dry cough and dypsnea on exertion that had been ongoing for 2 months. A chest radiography showed multiple and variable-sized cystic lesions, on both lungs and both interstitial infiltration and consolidation in both lower lung fields. Tests for autoantibody showed positive results of anti-nuclear antibody and anti-Ro/La antibody. The patient underwent a video assisted thoracoscopic surgery biopsy and pathologically confirmed LIP. We report the first known case of LIP-associated with primary Sjogren's syndrome in Korea.

Pulmonary manifestations of primary sjögren’s syndrome.

Sjögren's syndrome (SS) is a complex autoimmune exocrinopathy with multifactorial pathogenesis and multisystem manifestation. It is called primary Sjögren's syndrome (PSS) when the manifestations are seen without any other co-existent rheumatic diseases. The incidence of respiratory system involvement varies widely in the reported medical literature, partly due to lack of a universal agreement over the diagnostic criteria of the disease and the type of study methods employed. Respiratory system manifestations are protean; upper airway symptoms are very common and so is the complaint of dry cough. The PSS patients may develop interstitial lung diseases (ILDs) such as usual interstitial pneumonia (UIP), non-specific interstitial pneumonia (NSIP), lymphocytic interstitial pneumonia (LIP), bronchiolitis obliterans and organising pneumonia (BOOP), etc. They may also develop the whole spectrum of lymphoproliferative disorders of the lung ranging from LIP to follicular bronchiolitis, nodular lymphoid hyperplasia and low-grade lymphomas. Therapeutic options include symptomatic and supportive measures and corticosteroids as the mainstay of the treatment for ILDs occurring in these patients. In recent years, rituximab (anti-CD20) has emerged as a promising treatment for this disease, though data from controlled trials are still lacking. Pulmonary involvement may be a source of significant morbidity in these patients, though only rarely, it is the cause of death.