HRCT of the chest at 2?months after initiating ART showed improvement in interstitial abnormalities of the lung (Fig.?3c). transbronchial-biopsy proven T-cellular interstitial lung disease with granulomas. Microbiological examinations did not reveal an etiologic agent. The patient was also diagnosed with HIV-associated vacuolar encephalomyelopathy on the basis of an elevated HIV viral load in cerebrospinal LG-100064 fluid. After initiating ART, the brain lesions and paraplegia improved significantly, and interstitial abnormalities of the lungs and cough disappeared. Conclusion This report highlights that even in the post-ART era in developed countries with advanced healthcare services, HIV-associated vacuolar encephalomyelopathy should be considered in the differential diagnosis of a progressive neurological disorder during the first visit. Furthermore, GLILD may represent an HIV-associated pulmonary manifestation that can be treated by ART. and bacteria. Cytology of CSF was negative. The T2-weighed transverse spinal MRI (Fig.?1) showed focal demyelinating lesions of the spinal cord from the 4th through the 7th vertebral body, and diffuse atrophies of the spinal cord. Axial FLAIR MR images of the brain (Fig.?2a) showed confluent white matter hyperintensity and diffuse brain atrophies, whereas no brain mass lesions were detected. Chest X-ray indicated bilateral reticulonodular opacities predominantly in the lower lung zones. High-resolution computed tomography (HRCT) of the chest showed multiple centrilobular small nodules and branching opacities within all lung lobes, which were associated with small areas of ground-glass opacity (GGO) in the LG-100064 peribronchiolar region and bronchial wall thickening (Fig.?3a). A mosaic pattern was noted on expiratory HRCT image (Fig.?3b) showing air-trapping in the small airways. Mild mediastinal lymphadenopathies were also observed. A pulmonary function test revealed Rabbit Polyclonal to CAGE1 vital capacity of 4.25?L (103.7%), forced expiratory volume in 1.0?s/forced vital capacity of 78.9%, LG-100064 and decreased diffuse capacity for carbon monoxide of 57.9%. Bronchoalveolar lavage (BAL), and transbronchial lung biopsy (TBLB) were conducted on day 10 of admission. The number of cells in the BAL fluid (BALF) was 5.0??105/mL, with a cell differentiation of alveolar macrophages (18.5%), neutrophils (8.4%), lymphocytes (46.6%), and eosinophils (26.5%). The CD4/8 ratio of BALF was 0.05; cultures for bacteria and mycobacteria including tuberculosis were negative; and PCR test results using BALF for HSV, CMV, and were all negative. Hematoxylin and eosin staining of the TBLB specimen showed marked lymphoid infiltrate in the alveolar septa (Fig.?4a). Occasional non-necrotizing granulomas composed of epithelioid histiocytes were found in the lung field (Fig.?4b). Among lung-infiltrating lymphocytes, T cells were dominant (Fig.?4b) with a relatively higher number of CD8 cells than CD4 cells. Neither dense fibrosis nor microorganisms were found on Elastin van Gieson, Grocott, or ZiehlCNeelsen staining. There were no CMV inclusion bodies or toxoplasma cysts in either the TBLB lung specimen or BALF cytology. Based on these findings, the patient was diagnosed with HIV-associated vacuolar encephalomyelopathy and HIV-associated GLILD. Open in a separate window Fig.?1 Spine MRI on admission. The sagittal T2-weighed MR image of the spine shows focal demyelinating lesions of the spinal cord from the 4th through the 7th vertebral body (arrowhead), and diffuse spinal atrophy Open in a separate window Fig.?2 Axial FLAIR brain MR image on admission (a) and post-ART (b). a Symmetrical and diffuse cortical and central atrophies and an extensive high signal of the white matter were detected. b A decrease in white matter signals compared to pre-ART Open in LG-100064 a separate window Fig.?3 Axial HRCT image of the chest on admission (a, b) and post-ART (c). a Multiple centrilobular small nodules and branching opacities within all lung lobes, associated with small areas of ground-glass opacities (GGO) in the peribronchiolar region and bronchial wall thickening are seen. b Axial HRCT image on expiratory scan (lower image) shows lobular and subsegmental areas of mosaic pattern (arrowheads) due to air trapping in small airways, which is not evident from the inspiratory scan (upper image). c Improvement in interstitial abnormalities of the lung compared to pre-ART Open in another windowpane Fig.?4 Histopathological top features of the TBLB specimen. a Marked lymphocyte infiltration without developing lymphoid follicle can be determined in the alveolar LG-100064 septa (hematoxylin and eosin [H&E] staining, magnification 200. Size pub?=?100 micro m). b Histiocytes with hinged nuclei are aggregated to create non-necrotizing granuloma (arrow mind, H&E, magnification 400. Size pub?=?50 micro m). c A lot of the lung-infiltrated lymphocytes are Compact disc3-positive T cells (remaining, Compact disc3; right, Compact disc20, both magnification 40, Size pub?=?500 micro m) Treatment with emtricitabine/tenofovir alafenamide fumarate and dolutegravir and prophylaxis with 1?g of trimethoprim-sulfamethoxazole.