Serum KL-6 reflects alveolar damage and regeneration of type II pneumocytes, indicating disease activity in various interstitial lung diseases. We conducted a descriptive and observational multiple case-control study to determine the distribution of serum KL-6 levels in pediatric patients with or without respiratory diseases. Subjects were recruited from the patients of a teaching hospital in the suburb of Tokyo. A consecutive series of 401 children (0-16 years old) underwent blood sampling for many clinical reasons. They comprised the following four groups: pneumonia (n = 96), bronchial asthma (n = 101), measles (n = 102), and nonrespiratory diseases (n = 102) as a control group. Standard upper limits of serum KL-6 in a group of children with nonrespiratory disease were 250 U/mL, or half the adult level. No gender or age differences were observed. Elevated serum KL-6 concentrations were observed in severe pneumonia, acute exacerbations of asthma, and measles pneumonia. In the measles group, KL-6 values reflected the presence and severity of complicating pneumonia. We conclude that serum KL-6 levels exceeding 250 U/mL were rarely observed in children without respiratory diseases. In contrast, a substantial proportion of children with common respiratory diseases showed mild to moderate increases in serum KL-6 levels. Elevated serum KL-6 in these children may reflect the presence of alveolar damage, followed by regeneration of type II pneumocytes. However, in order to use serum KL-6 as a marker of interstitial lung diseases in children, a cutoff level should be determined separately.
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http://dx.doi.org/10.1002/ppul.10044 | DOI Listing |
BMC 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.
Rheumatology (Oxford)
December 2024
Department of Dermatology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan.
Objectives: Rituximab is emerging as a promising therapeutic option for systemic sclerosis-associated interstitial lung disease (SSc-ILD). However, little is known about factors that predict the efficacy of rituximab in SSc-ILD.
Methods: A post-hoc analysis was performed on prospective data from 48 patients with SSc-ILD in the double-blind, randomized, placebo-controlled DESIRES trial.
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