Pediatr Allergy Respir Dis.  2009 Sep;19(3):307-313.

A Case of Epstein-Barr-Virus-Associated Lymphocytic Interstitial Pneumonia

• Kim JH
• Oh SH
• Song YW
• Kim BJ
• Yu JH
• Hwang JE
• Hong SJ

Abstract

Lymphocytic interstitial pneumonia (LIP) is a benign lymphoproliferative disorder characterized histologically by diffuse infiltration with predominantly mature lymphocytes, plasma cells and reticuloendothelial cells in the alveolar septae and along the lymphatic vessels. LIP is an uncommon disease frequently associated with autoimmune diseases such as Sj?gren's syndrome, systemic lupus erythematosus, chronic active hepatitis, thyroiditis, myasthenia gravis, autoimmune hemolytic anemia and Human Immunodeficiency Virus (HIV) or Epstein- Barr virus (EBV) infection. LIP can progress to pulmonary or systemic lymphoma. LIP in children has variable disease courses such as spontaneous resolution, episodic worsening or progression to frank respiratory failure. Intravenous immunoglobulin and corticosteroids yield variable results, and some children may respond to them for several years. We report herein a case of EBV-associated, HIV-negative LIP diagnosed by thoracoscopic lung biopsy and a clonality study in a 5 year-old boy who was admitted with recurrent coughing and dyspnea. The patient was treated and fully recovered with methylprednisolone and high dose acyclovir. At the 3-year follow-up, he showed no clinical symptom despite increased EBV copy in bronchoalveolar lavage and progressive bronchiectasis in the right middle lobe.

Keyword

EBV-associated Lymphocytic Interstitial Pneumonia; child

MeSH Terms

Acyclovir
Adrenal Cortex Hormones
Anemia, Hemolytic, Autoimmune
Autoimmune Diseases
Biopsy
Bronchiectasis
Bronchoalveolar Lavage
Child
Coat Protein Complex I
Cough
Dyspnea
Follow-Up Studies
Hepatitis, Chronic
Herpesvirus 4, Human
HIV
Humans
Immunoglobulins
Lip
Lung
Lung Diseases, Interstitial
Lupus Erythematosus, Systemic
Lymphatic Vessels
Lymphocytes
Lymphoma
Lymphoproliferative Disorders
Methylprednisolone
Myasthenia Gravis
Plasma Cells
Respiratory Insufficiency
Thyroid Gland
Thyroiditis
Viruses
Acyclovir
Adrenal Cortex Hormones
Coat Protein Complex I
Immunoglobulins
Methylprednisolone