Human immunodeficiency virus-associated vacuolar encephalomyelopathy with granulomatous-lymphocytic interstitial lung disease improved after antiretroviral therapy: a case report

• Published in: AIDS research and therapy 2020-07, Vol.17 (1), p.1-38, Article 38
• Main Authors: Akagi, Kazumasa, Yamamoto, Kazuko, Umemura, Asuka, Ide, Shotaro, Hirayama, Tatsuro, Takazono, Takahiro, Imamura, Yoshifumi, Miyazaki, Taiga, Sakamoto, Noriho, Shiraishi, Hirokazu, Takahata, Hideaki, Zaizen, Yoshiaki, Fukuoka, Junya, Morikawa, Minoru, Ashizawa, Kazuto, Teruya, Katsuji, Izumikawa, Koichi, Mukae, Hiroshi
• Format: Article
• Language: English
• Subjects: Abnormalities; Acquired immune deficiency syndrome; AIDS; AIDS/HIV; Alveoli; Antigens; Antiretroviral agents; Antiretroviral drugs; Antiretroviral therapy; Biopsy; Blood tests; Brain; Bronchus; Care and treatment; Case Report; Case reports; Case studies; Catheters; Cellular biology; Cerebrospinal fluid; Common variable immunodeficiency; Complications and side effects; Cough; Cytomegalovirus; Demyelination; Developed countries; Diagnosis; Differential diagnosis; Drug therapy; Encephalopathy; Etiology; Granulomatous-lymphocytic interstitial lung disease; Hepatitis; HIV; HIV infections; Human immunodeficiency virus; Interstitial lung diseases; Lesions; Lung diseases; Lymphocytes; Lymphocytes T; Lymphocytosis; Neurological diseases; Paraplegia; Paraplegics; Patients; Spinal cord; Substantia alba; Tuberculosis; Vacuolar myelopathy; Viruses
• ISSN: 1742-6405; 1742-6405
• DOI: 10.1186/s12981-020-00295-y

Background Vacuolar encephalomyelopathy, a disregarded diagnosis lately, was a major neurological disease in the terminal stages of human immunodeficiency virus (HIV)-1 infection in the pre-antiretroviral therapy (ART) era. Granulomatous-lymphocytic interstitial lung disease (GLILD) was classically identified as a non-infectious complication of common variable immunodeficiency; however, it is now being recognized in other immunodeficiency disorders. Here, we report the first case of GLILD accompanied by vacuolar encephalomyelopathy in a newly diagnosed HIV-infected man. Case presentation A 40-year-old Japanese man presented with chronic dry cough and progressing paraplegia. Radiological examination revealed diffuse pulmonary abnormalities in bilateral lungs, focal demyelinating lesions of the spinal cord, and white matter lesions in the brain. He was diagnosed with GLILD based on marked lymphocytosis detecting in bronchoalveolar lavage, and 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 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. Keywords: HIV, AIDS, Encephalopathy, Vacuolar myelopathy, Granulomatous-lymphocytic interstitial lung disease, Antiretroviral therapy