Lymphoid interstitial pneumonitis
Noah Lechtzin, M.D.
- LIP is common cause of chronic respiratory disease in HIV+ African children, but no studies published from Zambia.
- Since LIP was not recognized clinical problem prior to the HIV epidemic, health workers may have little knowledge of the condition; often misdiagnosed as miliary or pulmonary TB.
- Several reports (from S. Africa, Zimbabwe, Rwanda, Malawi) of HIV+ children with chronic respiratory symptoms found LIP to be the most common Dx.
- Children with LIP usually present after 2 years of age; associated clinical features include generalized lymphadenopathy, bilateral nontender parotid enlargement, digital clubbing, and marked hepatomegaly.
- Secondary bacterial infection/pneumonia due to S. pneumoniae or Salmonella is common.
- Corticosteroids are useful in alleviating symptoms, but prophylaxis or even treatment for pulmonary TB should also be considered.
Zambia Information Author: David Riedel, M.D.
- Diffuse idiopathic interstitial lung disease; key pathologic finding: polyclonal lymphoid cell infiltrate of alveolar septae
- Occurs in <1% of HIV+ adults but up to 40% HIV+ children
- Sx: gradual onset dyspnea, cough, and fever
- May be asymptomatic, especially adults
- Less common Sx: weight loss, pleuritic pain, arthralgia
- PE: basilar crackles; children may have adenopathy & clubbing
- Approximately 2/3 of cases are indolent but 1/3 progress more rapidly
- CXR: bilateral, reticulonodular densities and or small nodules
- High resolution CT: bilateral ground glass infiltrates and nodules (2-4 mm), occasionally cysts
- In adults surgical lung biopsy usually necessary for diagnosis
- Children may be diagnosed empirically if CXR abnormalities persist >2 mos and no evidence of infectious cause (usually by bronchoscopic exclusion).
- Frequently mimics PCP and may be misdiagnosed clinically
- Children have been treated with prednisone 2 mg/kg once-daily x 2-4 wks followed by taper
- Adults should receive 1 mg/kg once-daily of prednisone x 8-12 wks, followed by taper over 6-8 wks to 0.25mg/kg
- Data on treatment limited and optimal duration unknown, but 6-12 mos common
- Some pts require life-long low-dose corticosteroids (e.g. prednisone 0.25 mg/kg once-daily)
- Case reports of children improving with antiviral therapy (acyclovir 1,500 mg/m2 daily) directed at EBV
- May improve with institution of HAART
- Resolves with 6-12 mos of corticosteroid treatment in some, but others may require life-long low-dose corticosteroid therapy
- Obtain serial thoracic CT and pulmonary function tests every 2-3 mos to assess initial response to treatment
- Decrease frequency of follow up if disease improves or stabilizes