Lymphoid interstitial pneumonia
Updates to Article Attributes
Lymphocytic interstitial pneumonitis (LIP) is a benign lymphoproliferative disorder characterised by lymphocyte predominant infiltration of the lungs 1,5. It is classified as a subtype of interstitial lung disease. It also falls under the umbrella of non lymphomatous pulmonary lymphoid disorders 7.
Epidemiology
LIP can occur at any age; however,most of patients are adults with mean age of 50 years. If a child presents with LIP, it iscan be indicative of AIDS.
There is a recognised female predilection most likely attributable to the fact that LIP occurs in patients with autoimmune disease such as Sjogren which is by far more common in females 8.
Clinical presentation
The main clinical symptoms are gradual onset of dyspnea and cough with approximately 6 months duration. Less frequently, patients may have systemic symptoms such as fever, night sweat, arthralgia, and weight loss. If disease progress to the end-stage respiratory failure cyanosis and clubbing may develop.
Hypertrophy of the salivary glands observed in 20% of patients.11
In about 80% of patients polyclonal or IgM monoclonal gammopathy is found.8
Pathology
There is generally diffuse infiltration of the interstitium and alveolar spaces by lymphocytes and plasma cells.
Associations
- Sjogren syndrome - can occur in up to 25% of those with LIP 6
- AIDS - particularly if it occurs in the young 9
- auto immune thyroid disease
- systemic lupus erythematosus (SLE)
- Castleman disease
- common variable immune deficiency (CVID)10
- rheumatoid arthritis
Radiographic features
Chest radiograph
- features can be non specific, but may include
- lower-zone predominant bilateral reticular opacification
- chronic bilateral airspace opacification
HRCT
The following features may be seen with LIP on HRCT, but the findings are not exclusive to its diagnosis.
- features generally tend to be diffuse with mid lower lobe predominance
- thickening of bronchovascular bundles
- intersitital thickening along lymph channels 2
- small but variably sized pulmonary nodules (can be centrilobular or subpleural, and often ill defined)
- ground-glass change
- scattered thin walled cysts - usually deep within the lung parenchyma- perivascular or sub pleural- typically a cyst abuts a vessel
. Size; size range from 1-30 mm (useful for differentiation between lymphoma or 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 end stage disease and honey combing despite treatment.
Transformation to lymphoma can occur particularly in patient with monoclonal gammopathy or hypogammaglobulinemia.8
Corticosteroids have been successfully trialled 1.
Differential diagnosis
General imaging differential considerations include
- pneumocystis pneumonia (PCP/PJP) - can be difficult to differentiate particularly in those with AIDS
See also
-<p><strong>Lymphocytic interstitial pneumonitis (LIP) </strong>is a benign lymphoproliferative disorder characterised by lymphocyte predominant infiltration of the lungs <sup>1,5</sup>. It is classified as a subtype of <a href="/articles/idiopathic-interstitial-pneumonias">interstitial lung disease</a>. It also falls under the umbrella of <a href="/articles/non-lymphomatous-pulmonary-lymphoid-disorders">n</a><a href="/articles/non-lymphomatous-pulmonary-lymphoid-disorders">on lymphomatous pulmonary lymphoid disorders</a> <sup>7</sup>.</p><h4>Epidemiology</h4><p>LIP can occur at any age; however,most of patients are adults with mean age of 50 years. If a child presents with <strong>LIP</strong>, it is indicative of <strong>AIDS</strong>.</p><p>There is a recognised female predilection most likely attributable to the fact that LIP occurs in patients with autoimmune disease such as Sjogren which is by far more common in females <sup>8</sup>.</p><h4>Clinical presentation</h4><p>The main clinical symptoms are gradual onset of dyspnea and cough with approximately 6 months duration. Less frequently, patients may have systemic symptoms such as fever, night sweat, arthralgia, and weight loss. If disease progress to the end-stage respiratory failure cyanosis and clubbing may develop.</p><p>Hypertrophy of the salivary glands observed in 20% of patients.<sup>11</sup></p><p>In about 80% of patients polyclonal or IgM monoclonal gammopathy is found.<sup>8</sup></p><h4>Pathology </h4><p>There is generally diffuse infiltration of the interstitium and alveolar spaces by lymphocytes and plasma cells.</p><h5>Associations</h5><ul>- +<p><strong>Lymphocytic interstitial pneumonitis (LIP) </strong>is a benign lymphoproliferative disorder characterised by lymphocyte predominant infiltration of the lungs <sup>1,5</sup>. It is classified as a subtype of <a href="/articles/idiopathic-interstitial-pneumonias">interstitial lung disease</a>. It also falls under the umbrella of <a href="/articles/non-lymphomatous-pulmonary-lymphoid-disorders">n</a><a href="/articles/non-lymphomatous-pulmonary-lymphoid-disorders">on lymphomatous pulmonary lymphoid disorders</a> <sup>7</sup>.</p><h4>Epidemiology</h4><p>LIP can occur at any age; however,most of patients are adults with mean age of 50 years. If a child presents with <strong>LIP</strong>, can be indicative of <strong>AIDS</strong>.</p><p>There is a recognised female predilection most likely attributable to the fact that LIP occurs in patients with autoimmune disease such as Sjogren which is by far more common in females <sup>8</sup>.</p><h4>Clinical presentation</h4><p>The main clinical symptoms are gradual onset of dyspnea and cough with approximately 6 months duration. Less frequently, patients may have systemic symptoms such as fever, night sweat, arthralgia, and weight loss. If disease progress to the end-stage respiratory failure cyanosis and clubbing may develop.</p><p>Hypertrophy of the salivary glands observed in 20% of patients.<sup>11</sup></p><p>In about 80% of patients polyclonal or IgM monoclonal gammopathy is found.<sup>8</sup></p><h4>Pathology </h4><p>There is generally diffuse infiltration of the interstitium and alveolar spaces by lymphocytes and plasma cells.</p><h5>Associations</h5><ul>
-<li>scattered thin walled cysts - usually deep within the lung parenchyma- perivascular or sub pleural- typically a cyst abuts a vessel. Size range from 1-30 mm (useful for differentiation between lymphoma or the lung <sup>1</sup>)</li>- +<li>scattered thin walled cysts - usually deep within the lung parenchyma- perivascular or sub pleural- typically a cyst abuts a vessel; size range from 1-30 mm (useful for differentiation between lymphoma or the lung <sup>1</sup>)</li>
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