Discussion
Diagnosis With Brief Discussion
- Diagnosis
- Lymphocytic interstitial pneumonia in a patient with SLE
- Radiologic Findings
- Fig 1. Diffuse ill-defined increased opacity in both mid to lower lung zones on chest radiograph.
Fig 2-6. CT images show diffuse and bilateral ground glass opacity lesions and multiple thin walled and well-demarcated cysts in both lungs.
- Brief Review
- The lymphocytic interstitial pneumonia (LIP) is a rare disease characterized by diffuse infiltration of lymphocytes and plasma cells within the lung parenchyma. In the adult population, it most commonly affects middle age women, frequently occurring in patients with Sjögren syndrome, autoimmune thyroid disease, myasthenia gravis, and pernicious anemia. One-third of adult cases of LIP is associated with Sjögren Syndrome.
The thorax is more frequently involved in SLE than in any other collagen vascular disease. The majority of patients with SLE have thoracic involvement at some time during their clinical course. Pleural and pericardial disease are the most common thoracic manifestations. Infectious pneumonia, lupus pneumonitis, alveolar hemorrhage, chronic interstitial pneumonitis/fibrosis, pulmonary hypertension, thromboembolism, bronchiolitis obliterans, bronchiolitis obliterans organizing pneumonia, and diaphragmatic muscle dysfunction have been described. Although there has been prior association between SLE and pulmonary lymphoma, LIP is distinctly uncommon in SLE. The majority of patients with SLE will develop thoracic involvement at some point in their disease. This is most frequently pleural and pericardial disease. LIP, although commonly associated with autoimmune disease, is rarely seen in patients with SLE.
Reflecting diffuse infiltration of lymphocytes and plasma cells, HRCT usually reveals thickening of perilymphatic interstitium, ground-glass opacity, and poorly defined centrilobular nodules. Furthermore, multiple variable sized thin-walled cystic airspaces are common finding, and should be good differential point from other idiopathic interstitial pneumonias. Other findings include lymph node enlargement (in about 50% of cases) and bronchiectasis.
Definite diagnosis can be made with lung biopsy. Although there is no standard treatment for LIP, steroid therapy is commonly used and other immunosuppressive drugs may be combined.
Prognosis is variable, and can be grave. Therefore, keen radiologic suspicion and early diagnosis is crucial.
- References
- 1. T Johkoh, N Muller, H Pickford, et al. Lymphocytic interstitial pneumonia: thin-section CT findings in 22 patients. Radiology 1999;212:567.
2. Filipek S, Thompson ME, Wang PL, et al. J Thorac Imaging. 2004 Jul;19(3):200-3. Lymphocytic interstitial pneumonitis in a patient with systemic lupus erythematosus: radiographic and high-resolution CT findings.
- Keywords
- Lung, Interstitial pneumonia, Systemic lupus erythematosus,