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Interstitial lung disease (ILD) has been identified as a prevalent complication and significant contributor to mortality in individuals with pemphigus. In this study, a murine model of pemphigus was developed through the subcutaneous administration of serum IgG obtained from pemphigus patients, allowing for an investigation into the association between pemphigus and ILD. Pulmonary interstitial lesions were identified in the lungs of a pemphigus mouse model through histopathology, RT-qPCR and Sircol assay analyses. The severity of these lesions was found to be positively associated with the concentration of IgG in the injected serum. Additionally, DIF staining revealed the deposition of serum IgG in the lung tissue of pemphigus mice, indicating that the subcutaneous administration of human IgG directly impacted the lung tissue of the mice, resulting in damage. This study confirms the presence of pulmonary interstitial lesions in the pemphigus mouse model and establishes a link between pemphigus and ILD.
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http://dx.doi.org/10.1111/exd.15136 | DOI Listing |
Mod Rheumatol
September 2025
Chugai Pharmaceutical Co., Ltd., 1-1 Nihonbashi-Muromachi 2-Chome, Chuo-ku, Tokyo 103-8324, Japan.
ObjectivesThe 2023 EULAR guidelines for systemic sclerosis (SSc) newly recommend biologics (rituximab, tocilizumab), mycophenolate mofetil (MMF), and nintedanib in addition to cyclophosphamide for interstitial lung disease (ILD). This study investigated recent actual use of these drugs in Japan. MethodsWe analysed data from a Japanese hospital claims database (2020-2023), identifying patients with SSc disease codes (ICD-10 M34.
View Article and Find Full Text PDFEur J Case Rep Intern Med
July 2025
Servicio de Alergia e Inmunología, Hospital Británico de Buenos Aires, Argentina.
Introduction: Interstitial lung disease is a major complication in patients with common variable immunodeficiency. There are some publications that try to shed light on the pathophysiology of this non-infectious complication, most of them highlight the role of follicular T cells and CD21 B cells. Moreover, there are no guidelines based on randomized controlled studies on the treatment of patients with interstitial lung disease and the published case series or small uncontrolled studies describe a wide range of response rates to treatment.
View Article and Find Full Text PDFFront Pediatr
August 2025
Pediatrics Department, Kazan State Medical University of the Ministry of Health of the Russian Federation, Kazan, Russia.
Background: Acid sphingomyelinase deficiency (ASMD) type A/B, a rare lysosomal storage disorder caused by biallelic mutations in the SMPD1 gene, presents with variable visceral and neurological manifestations. Arnold-Chiari malformation is a structural defect of the cerebellum and brainstem with distinct pathogenesis and clinical course. To our knowledge, the coexistence of these two conditions has not been previously reported.
View Article and Find Full Text PDFChron Respir Dis
September 2025
Department of Pulmonology, II.Medical Clinic and Polyclinic, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
Case presentationDescription of a patient with a progressive destructive lung disease resembling pleuroparenchymal fibroelastosis, liver cirrhosis and bone marrow changes. Genetic workup identified a rare heterozygous coding variant in the (telomerase reverse transcriptase) gene c.472 C>T; p.
View Article and Find Full Text PDFVirchows Arch
September 2025
Department of Anatomic Pathology, Tohoku University Graduate School of Medicine, Sendai, Japan.
Lung adenocarcinoma (LUAD) associated with usual interstitial pneumonia (UIP) harbours distinct features compared to lung adenocarcinoma without UIP. Therefore, we aimed to characterise the tumour microenvironment of LUAD with UIP by focusing on cancer-associated fibroblasts (CAFs) and stromal composition. Immunohistochemistry was performed on 32 LUAD samples (16 each with and without UIP) to evaluate CAF marker expression and lymphocyte infiltration.
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