Idiopathic Lymphocytic Interstitial Pneumonia

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

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

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