eMedicine Specialties > Pulmonology > Interstitial Lung Diseases

Lymphocytic Interstitial Pneumonia: Differential Diagnoses & Workup

Author: Jussi J Saukkonen, MD, Assistant Professor, Department of Internal Medicine, Division of Pulmonary/Critical Care Medicine, Boston University School of Medicine, Boston Medical Center
Contributor Information and Disclosures

Updated: Jan 17, 2008

Differential Diagnoses

Acute Respiratory Distress Syndrome
Pneumonia, Fungal
Hypersensitivity Pneumonitis
Pneumonia, Viral
Lymphomatoid Granulomatosis
Pulmonary Edema, Cardiogenic
Miliary tuberculosis
Pulmonary Fibrosis, Idiopathic
Pneumocystis Carinii Pneumonia
Pulmonary Fibrosis, Interstitial (Nonidiopathic)
Pneumonia, Bacterial
Sarcoidosis

Other Problems to Be Considered

Angioimmunoblastic lymphadenopathy
Benign lymphocytic angiitis
Granulomatosis
Nonspecific interstitial pneumonitis
Plasma cell interstitial pneumonitis
Interstitial lung disease

Workup

Laboratory Studies

  • Laboratory tests are nonspecific.
  • The most essential items are the chest radiograph, measurement of gas exchange, and histology.
  • Serum protein electrophoresis: Polyclonal hypergammaglobulinemia is common.
  • Lactate dehydrogenase
    • In pediatric patients with LIP and HIV, lactate dehydrogenase (LDH) may be elevated to 300-500 IU/L, approximately half the levels seen in Pneumocystis carinii pneumonia.
    • This measurement is not helpful in adults.
  • Serologic testing: Test for HIV type 1, HTLV type 1, EBV, and rheumatoid factor.

Imaging Studies

  • Chest radiograph
    • Bibasilar interstitial or micronodular infiltrates (see Media File 1) with coalescence into an alveolar pattern are present.
    • In adults, honeycombing is present in up to one third of cases. Hilar adenopathy and pleural effusion are uncommon.
    • Similar infiltrates are seen in children, often with mediastinal widening and hilar enlargement denoting pulmonary lymphoid hyperplasia.
  • CT scan
    • This reveals the extent of the disease.
    • It may demonstrate bronchiectasis.
    • It also demonstrates the degree of fibrosis.
    • Findings may be used to follow disease progression.
    • Long-term follow-up may show the development of fibrosis, bronchiectasis, micronodules, bullae, and/or cystic changes.

Other Tests

  • Pulmonary function testing
    • This usually demonstrates restriction with a reduced or normal diffusion capacity.
    • Obstructive airway disease has been reported occasionally.
  • Arterial blood gas measurement: This may be helpful in assessing the severity of illness, but the findings are nonspecific. Findings include the following:
    • Partial pressure of oxygen (PO2) measurement is normal.
    • Profound hypoxemia and/or an increased alveolar to arterial (A-a) oxygen gradient is present.
    • Pulse oximetry is used for screening, but it may not detect an A-a gradient. It should be checked at rest and following exercise.

Procedures

  • Bronchoscopy with transbronchial biopsy
    • Generally, this is diagnostic if multiple biopsies are obtained from several affected subsegments.
    • Exact sensitivity and specificity of transbronchial biopsy is not reported.
    • Open lung biopsy is the criterion standard. It may be required in the face of nonspecific or equivocal findings, as with extensive fibrosis.

Histologic Findings

Histology shows alveolar septal and intra-alveolar infiltration by small, mature, noncleaved polyclonal lymphocytes and plasma cells. Lymphoid follicles or micronodules also may be present. No intrapulmonary lymphadenopathy, vasculitis, or necrosis is observed. Extensive areas of interstitial fibrosis may be present. Noncaseating granulomata have been reported.

More on Lymphocytic Interstitial Pneumonia

Overview: Lymphocytic Interstitial Pneumonia
Differential Diagnoses & Workup: Lymphocytic Interstitial Pneumonia
Treatment & Medication: Lymphocytic Interstitial Pneumonia
Follow-up: Lymphocytic Interstitial Pneumonia
Multimedia: Lymphocytic Interstitial Pneumonia
References
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References

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Further Reading

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Keywords

lymphocytic interstitial pneumonia, LIP, lymphoid interstitial pneumonitis, lymphoid pneumonitis, plasma cell interstitial pneumonitis, pulmonary interstitial infiltration, pseudolymphoma, autoimmune disorders, lymphoproliferative disorders, human immunodeficiency virus, HIV-related LIP, HIV-associated LIP, HIV, AIDS, Epstein-Barr virus, EBV, human T-cell leukemia virus, HTLV type 1, HIV type 1, rheumatoid arthritis, Hashimoto thyroiditis, myasthenia gravis, pernicious anemia, autoerythrocyte sensitization syndrome, chronic active hepatitis, common variable immunodeficiency, Sjögren syndrome, allogeneic bone marrow transplantation, lupus, lymphoma, B-cell CLL/lymphoma 6, BCL-6, zinc finger protein 51

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Contributor Information and Disclosures

Author

Jussi J Saukkonen, MD, Assistant Professor, Department of Internal Medicine, Division of Pulmonary/Critical Care Medicine, Boston University School of Medicine, Boston Medical Center
Jussi J Saukkonen, MD is a member of the following medical societies: American Thoracic Society
Disclosure: Nothing to disclose.

Medical Editor

Stephen P Peters, MD, PhD, Professor, Department of Medicine, Wake Forest University
Stephen P Peters, MD, PhD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Association of Immunologists, American College of Chest Physicians, American College of Physicians, American Federation for Medical Research, American Thoracic Society, and Sigma Xi
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Daniel R Ouellette, MD, FCCP, Associate Professor of Medicine, Wayne State University School of Medicine; Consulting Staff, Pulmonary Disease and Critical Care Medicine Service, Henry Ford Health System
Daniel R Ouellette, MD, FCCP is a member of the following medical societies: American College of Chest Physicians and American Thoracic Society
Disclosure: Boehringer Ingleheim Honoraria Speaking and teaching; Pfizer Honoraria Speaking and teaching

CME Editor

Timothy D Rice, MD, Associate Professor, Departments of Internal Medicine and Pediatrics and Adolescent Medicine, Saint Louis University School of Medicine
Timothy D Rice, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Physicians
Disclosure: Nothing to disclose.

Chief Editor

Zab Mosenifar, MD, Director, Division of Pulmonary and Critical Care Medicine, Director, Women's Guild Pulmonary Disease Institute, Executive Vice Chair, Department of Medicine, Cedars Sinai Medical Center; Professor of Medicine, David Geffen School of Medicine at UCLA
Zab Mosenifar, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, American Federation for Medical Research, and American Thoracic Society
Disclosure: Nothing to disclose.

 
 
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