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
