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Lymphoid Interstitial Pneumonia

(Lymphoid Interstitial Pneumonitis)

By

Joyce Lee

, MD, MAS, University of Colorado School of Medicine

Reviewed/Revised Jul 2023
VIEW PROFESSIONAL VERSION

Lymphoid interstitial pneumonia is an uncommon lung disease in which mature lymphocytes (a type of white blood cell White Blood Cells The main components of blood include Plasma Red blood cells White blood cells Platelets read more White Blood Cells ) accumulate in the air sacs of the lungs (alveoli).

  • People usually cough and have difficulty breathing.

  • Diagnosis requires chest x-ray, computed tomography, pulmonary function tests, and often bronchoscopy, biopsy, or both.

  • Treatment involves corticosteroids, immunosuppressants, or both.

Lymphoid interstitial pneumonia is a form of idiopathic interstitial pneumonia Overview of Idiopathic Interstitial Pneumonias Idiopathic interstitial pneumonias are interstitial lung diseases that have no known cause that have some similarities in symptoms and how they affect the lungs. Some types of these diseases... read more . It can occur in children, usually those who have human immunodeficiency virus Human Immunodeficiency Virus (HIV) Infection in Children and Adolescents Human immunodeficiency virus (HIV) infection is a viral infection that progressively destroys certain white blood cells and makes people more vulnerable to other infections and some cancers... read more (HIV) infection or other immunodeficiencies. Lymphoid interstitial pneumonia can also occur in adults, often those with autoimmune disorders such as plasma cell disorders Overview of Plasma Cell Disorders Plasma cell disorders are uncommon. They begin when a single plasma cell multiplies excessively. The resulting group of genetically identical cells (called a clone) produces a large quantity... read more , Sjögren syndrome Sjögren Syndrome Sjögren syndrome is a common autoimmune connective tissue disorder and is characterized by excessive dryness of the eyes, mouth, and other mucous membranes. White blood cells can infiltrate... read more Sjögren Syndrome , Hashimoto thyroiditis Hashimoto Thyroiditis Hashimoto thyroiditis is chronic, autoimmune inflammation of the thyroid gland. Hashimoto thyroiditis results when antibodies in the body attack the cells of the thyroid gland—an autoimmune... read more , rheumatoid arthritis Rheumatoid Arthritis (RA) Rheumatoid arthritis is an inflammatory arthritis in which joints, usually including those of the hands and feet, are inflamed, resulting in swelling, pain, and often destruction of joints.... read more Rheumatoid Arthritis (RA) , and systemic lupus erythematosus Systemic Lupus Erythematosus (SLE) Systemic lupus erythematosus is a chronic autoimmune inflammatory connective tissue disorder that can involve joints, kidneys, skin, mucous membranes, and blood vessel walls. Problems in the... read more Systemic Lupus Erythematosus (SLE) (lupus). Women and girls are more commonly affected. Average age of affected adults is 54 years.

Symptoms of Lymphoid Interstitial Pneumonia

Children develop cough and decreased ability to exercise, and they may not grow and gain weight. Adults develop difficulty breathing and cough over months or, in some cases, years. Less common symptoms include weight loss, fever, joint pain, and night sweats.

Diagnosis of Lymphoid Interstitial Pneumonia

  • Chest computed tomography

Blood tests may reveal abnormalities in their blood proteins that can help establish the diagnosis. If not, lung biopsy is usually necessary.

Treatment of Lymphoid Interstitial Pneumonia

  • Corticosteroids or immunosuppressant medications

Treatment is with corticosteroids, immunosuppressant medications (such as azathioprine or cyclophosphamide), or both, but the effectiveness of these drugs is unknown.

NOTE: This is the Consumer Version. DOCTORS: VIEW PROFESSIONAL VERSION
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