Idiopathic Lymphocytic Interstitial Pneumonia

Chapters

 

DIAGNOSIS

SYMPTOMS3

  • Patients present with gradually increasing cough and dyspnea over 3 or more years
  • Patients occasionally present with:
    • Fever
    • Joint Pain
    • Chest Pain
    • Weight Loss
  • Crackles may be detected as the disease progresses

DIAGNOSTIC TESTS

Thorough clinical history to exclude known causes such as:3

  • Rheumatoid arthritis
  • Sjögren's syndrome
  • Hashimoto's disease
  • Pernicious anemia
  • Chronic active hepatitis
  • Systemic lupus erythematosus
  • Autoimmune hemolytic anemia
  • Primary biliary cirrhosis
  • Myasthenia gravis
  • Hypogammaglobulinemia
  • Human immunodeficiency virus

Routine laboratory tests and serum protein electrophoresis:1

  • Polyclonal gammopathy
  • Hypogammaglobulinemia

Pulmonary function tests4

  • Reduced FVC and FEV1
  • Elevated FEV1/FVC
  • Decreased DLCO

HRCT6

  • Features: Ground-glass opacities, perivascular cysts, septal thickening, centrilobular nodules
  • Distribution: Basilar predominance or diffuse

Lung biopsy is required for diagnosis in adults1

  • Diffuse infiltration of alveolar septa by lymphoid cells, lymphoid hyperplasia frequent6
  • Histopathology pattern of LIP is similar to diffuse lymphoid hyperplasia, nodular lymphoid hyperplasia, lymphoma and the patterns of organizing pneumonia (OP), NSIP, hypersensitivity pneumonitis, and UIP3
  • Immunohistochemistry (IHC) and flow cytometry must be done to distinguish from primary lymphomas — both B and T cells must be present in infiltrate for LIP diagnosis

Bronchoalveolar lavage should be performed to rule out infection1

  • Fluid reveals many lymphocytes but immunophenotyping should not reveal any clonality3

2 patterns on chest X-ray have been described3

  • Basilar with an alveolar component
  • Diffuse with associated honeycombing

See also

Man smiling

Test your knowledge

Take the test 

Bridging gaps in ILD education image

Bridging educational gaps in ILD

Learn more 

Interstitial lung disease in systemic sclerosis with a focus on chest CT

Media library

See media