Lymphocytic interstitial pneumonitis is a benign lymphoproliferative disorder characterized by lymphocyte predominant infiltration of the lungs. It is classified as a subtype of interstitial lung disease. It also falls under the umbrella of non-lymphomatous pulmonary lymphoid disorders.
Lymphocytic interstitial pneumonitis can occur at any age. However, most of the patients are adults with a mean age of 50 years. If a child presents with lymphocytic interstitial pneumonitis, this can be indicative of AIDS.
There is a recognized female predilection most likely attributable to the fact that lymphocytic interstitial pneumonitis occurs in patients with autoimmune disease such as Sjogren syndrome, which is by far more common in women 8.
The main clinical symptoms are a gradual onset of dyspnea and cough with approximately six months duration. Less frequently, patients may have systemic symptoms such as fever, night sweat, arthralgia, and weight loss. If the disease progresses to the end-stage respiratory failure cyanosis and clubbing may develop. Hypertrophy of the salivary glands observed in 20% of patients 11.
There is diffuse infiltration of the interstitium and alveolar spaces by lymphocytes and plasma cells.
- Sjogren syndrome: can occur in up to 25% of those with lymphocytic interstitial pneumonitis 6
- AIDS: particularly if it occurs in the young 9
- autoimmune thyroid disease
- systemic lupus erythematosus
- Castleman disease
- common variable immune deficiency 10
- rheumatoid arthritis
- pulmonary amyloidosis
In about 80% of patients polyclonal or IgM monoclonal gammopathy is found 8.
Features can be non-specific, but may include:
The following features may be seen with lymphocytic interstitial pneumonitis on HRCT, but the findings are not exclusive to its diagnosis:
- features tend to be diffuse with mid to lower lobe predominance
- thickening of bronchovascular bundles
- interstitial thickening along lymph channels 2
- small but variable sized pulmonary nodules (can be centrilobular or subpleural, and are often ill-defined)
- ground-glass changes
- scattered thin-walled cysts
- usually deep within the lung parenchyma
- typically abut vessels (i.e. is perivascular or subpleural)
- size range between 1-30 mm (useful for differentiation from lymphoma of the lung 1)
- mediastinal lymphadenopathy
Treatment and prognosis
The natural history is variable, from near-complete resolution to progressive disease. More than 30% of patients will develop the end-stage disease and honeycombing despite treatment.
Transformation to lymphoma can occur, particularly in a patient with monoclonal gammopathy or hypogammaglobulinemia 8. Corticosteroids have been successfully trialled 1.
General imaging differential considerations include:
pneumocystis pneumonia (PCP)
- cystic changes (pneumatoceles) seen in advanced disease
- can be difficult to differentiate particularly in those with AIDS
- occur in younger females
- cysts are generally uniformly distributed throughout the lungs
Langerhans cell histiocytosis
- bizarre cysts that spare the costophrenic angles
- upper lung zone predominant
- 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. AJR Am J Roentgenol (abstract) - Pubmed citation
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- 7. Glickstein M, Kornstein MJ, Pietra GG et-al. Nonlymphomatous lymphoid disorders of the lung. AJR Am J Roentgenol. 1986;147 (2): 227-37. AJR Am J Roentgenol (abstract) - Pubmed citation
- 8. Ferguson EC, Berkowitz EA. Lung CT: Part 2, The interstitial pneumonias-clinical, histologic, and CT manifestations. AJR Am J Roentgenol. 2012;199 (4): W464-76. doi:10.2214/AJR.10.7309 - Pubmed citation
- 9. Das S, Miller RF. Lymphocytic interstitial pneumonitis in HIV infected adults. Sex Transm Infect. 2003;79 (2): 88-93. Free text at pubmed - Pubmed citation
- 10. Cha SI, Fessler MB, Cool CD et-al. Lymphoid interstitial pneumonia: clinical features, associations and prognosis. Eur. Respir. J. 2006;28 (2): 364-9. doi:10.1183/09031936.06.00076705 - Pubmed citation
- 11. Dähnert W. Radiology Review Manual. Lippincott Williams & Wilkins. (2011) ISBN:1609139437. Read it at Google Books - Find it at Amazon
- 12. Stojan G, Baer AN, Danoff SK. Pulmonary manifestations of Sjögren's syndrome. Current allergy and asthma reports. 13 (4): 354-60. doi:10.1007/s11882-013-0357-9 - Pubmed