Lymphoid interstitial pneumonia (LIP) was first described in 1969 by Liebow and Carrington. LIP is associated with autoimmune disorders such as Sjogren’s syndrome, as well as infectious etiologies like Epstein-Barr and HIV. The initial presentation of LIP with HIV is described in children, but rare in adults. We present the case of LIP as the initial presentation of HIV in an adult.
A twenty-eight year-old male presented with fevers for five days along with progressive cough and dyspnea for a month. He had been initially treated one month prior with moxifloxacin when a chest x-ray revealed a left lower lobe infiltrate. Review of systems was significant for a twenty pound weight loss over four weeks. Social history noted current tobacco abuse(10 pack years) and no illicit drug use. He was homosexual with multiple partners and unsure of his HIV status. Vital signs included a temperature of 39°F, and pulse oximetry of 97% on room air. Physical exam was remarkable only for a chest exam revealing left basilar rales. Laboratory values revealed a white cell count of 10.9×109/L with a normal differential. Thoracic computed tomography revealed ground glass opacities and dense consolidation in the left lower lobe (Figure 1). An HIV antibody test was sent. Bronchoscopy with bronchoalveolar lavage and transbronchial biopsies of the left lower lobe was performed. Bacterial, fungal, viral, and mycobacterial cultures were negative. Cytological analysis revealed no evidence of pneumocystis or malignancy. Pathological examination of transbronchial biopsy revealed a prominent interstitial inflammatory infiltrate consisting of lymphocytes, macrophages, and occasional plasma cells (Figure 2) consistent with a diagnosis of LIP. Immunohistochemical staining of virtually all lymphocytes were positive for CD3, along with positive CD20 staining of the nodules for B lymphocytes. HIV antibody testing returned positive with a CD4 count of 110 cells/μL. He had symptomatic improvement with corticosteroids (0.5 mg/kg) at time of discharge and unfortunately has been lost to follow-up.
LIP was originally classified as a lymphoproliferative disorder limited to the lung. Many believed it was a precursor for pulmonary lymphoma; however, this appears unlikely as malignant transformation has been rarely reported. Histologically, diffuse alveolar infiltration with lymphocytes and other cellular elements, including plasma cells are present (1). LIP appears to be more common in middle-aged women, often being associated with diseases of immune dysfunction. Symptoms include cough, breathlessness, fevers, and weight loss. Chest exam usually reveals bibasilar rales (1). LIP is more common in children with HIV while rare in the adult HIV population, especially Caucasian homosexuals. LIP as an initial presentation of HIV in adults appears rare and has not been reported to our knowledge. LIP occurs more commonly in adults with HIV and normal CD4 counts, whereas non-specific interstitial pneumonia occurs in patients with CD4 counts <200 cells/μL (2 ). Treatment options for LIP are poorly defined and it is unclear whether treatment alters the natural course of the disease. Variable clinical courses have been described with corticosteroids; however, it appears patients with HIV associated LIP may have a more favorable response compared to others with LIP. Antiretroviral therapy has also been suggested as a treatment option as reports of disease remission after antiretroviral therapy alone are described (2 ).
LIP is uncommon in adults with HIV and rarely a presenting feature of HIV. Current treatment recommendations include antiretroviral therapy and/or corticosteroids, although the scientific evidence to back these recommendations is sparse. HIV testing should be offered to all patients presenting with a new diagnosis of LIP.
Christopher Gilbert, No Financial Disclosure Information; No Product/Research Disclosure Information