We report the case of a 42-year-old male who underwent allogeneic bone marrow transplantation (BMT) for acute myelogenous leukemia, and then developed pneumonitis with a bronchiolitis obliterans organizing pneumonia (BOOP)-like shadow. When he came with exertional dyspnea four months after BMT, the chest X-ray and CT findings disclosed bilateral infiltration, and remarkable elevation of his serum KL-6 level, a monitoring marker for disease activity in interstitial lung disease. Although organizing pneumonia (OP) was revealed by a transbronchial lung biopsy, no pathogen was detected in bacterial, fungal and routine viral cultures or by direct cytological examinations using bronchoalveolar lavage (BAL) specimens. Since human herpes virus-6 (HHV-6) was detected in BAL specimens by the polymerase chain reaction (PCR), a diagnosis of a pneumonitis-like BOOP shadow related to HHV-6 was made, and he was treated with methylprednisolone and ganciclovir (GCV). Although there was a relapse of his OP 1.5 months later, with re-elevation of his serum KL-6 level, continuous administration of GCV led to disappearance of HHV-6 in BAL specimens assayed by PCR, in association with normalization of the serum KL-6 level. HHV-6 should be considered as a cause of unexplained pneumonitis in BMT recipients, and KL-6 is useful for monitoring the pneumonitis status in these patients.
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http://dx.doi.org/10.11150/kansenshogakuzasshi1970.76.385 | DOI Listing |
Biomolecules
January 2025
Department of Respiratory Medicine, Juntendo University Faculty of Medicine and Graduate School of Medicine, Tokyo 113-8421, Japan.
Diffuse interstitial lung diseases (ILD) include conditions with identifiable causes such as chronic eosinophilic pneumonia (CEP), sarcoidosis (SAR), chronic hypersensitivity pneumonitis (CHP), and connective tissue disease-associated interstitial pneumonia (CTD), as well as idiopathic interstitial pneumonia (IIP) of unknown origin. In non-IIP diffuse lung diseases, bronchoalveolar lavage (BAL) fluid appearance is diagnostic. This study examines lymphocyte subsets in BAL fluid and peripheral blood of 56 patients with diffuse ILD, excluding idiopathic pulmonary fibrosis (IPF), who underwent BAL for diagnostic purposes.
View Article and Find Full Text PDFBMC Pulm Med
January 2025
Department of Pulmonary Medicine, National Hospital Organization MinamiKyushu Hospital, 1882 Kida, Aira-Shi, Kagoshima, 899-5293, Japan.
Background: Reports of autoimmune diseases coexisting with autoimmune pulmonary alveolar proteinosis (autoimmune PAP; APAP) are extremely rare. APAP coexisting with autoimmune diseases may often be misdiagnosed as connective tissue disease-associated interstitial lung disease (ILD). Here, we describe a rare case of a patient with systemic sclerosis who was diagnosed with APAP after the exacerbation of lung opacities during treatment with immunosuppressive agents.
View Article and Find Full Text PDFJ Rheumatol
January 2025
J.A. Sparks, MD, MMSc, Brigham and Women's Hospital, Division of Rheumatology, Inflammation, and Immunity and Harvard Medical School, Boston, Massachusetts, USA.
Objective: To investigate baseline and change of pulmonary damage biomarkers (serum Krebs von den Lungen 6 [KL-6], human surfactant protein D [hSP-D], and matrix metalloproteinase 7 [MMP-7]) with rheumatoid arthritis-associated interstitial lung disease (RA-ILD) progression.
Methods: In the Korean Rheumatoid Arthritis Interstitial Lung Disease (KORAIL) cohort, a prospective cohort, we enrolled patients with RA and ILD confirmed by chest computed tomography imaging and followed annually. ILD progression was defined as worsening in physiological and radiological domains of the 2022 American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Society guideline for progressive pulmonary fibrosis (PPF).
Immunobiology
January 2025
Department of Respiratory and Critical Care Medicine, Fujian Medical University Union Hospital, China.
Tumor immunotherapy, particularly immune checkpoint inhibitors (ICIs), has emerged as a powerful strategy in treating malignant tumors, exhibiting efficacy in both first-line and second-line treatments for advanced non-small cell lung cancer (NSCLC). Despite their success, ICIs can lead to adverse reactions, including interstitial lung disease (ILD), with an incidence ranging from 2.7 % to 20.
View Article and Find Full Text PDFEur J Med Res
January 2025
Department of Respiratory and Critical Care Medicine, Nanjing Drum Tower Hospital, Clinical College of Nanjing Medical University, Nanjing, Jiangsu, China.
Background: Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic lung disease with poor prognosis, nomogram model for its prognosis and acute exacerbation was constructed.
Methods: Two hundred and sixty eight patients with IPF were grouped with different severity according to fibrosis area, serum Club cell secretory protein 16(CC16) was compared between these groups. All patients were randomly divided into training and testing sets.
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