488 results match your criteria Collagen-Vascular Disease Associated With Interstitial Lung Disease

Anti-glomerular basement membrane disease associated with thin basement membrane nephropathy: A case report.

Medicine (Baltimore) 2021 May;100(20):e26095

Department of Internal Medicine.

Rationale: Simultaneous occurrence of anti-glomerular basement membrane (anti-GBM) disease and thin basement membrane nephropathy (TBMN), both of which invade the type IV collagen subunits, is very rare. Here, we present the case of a 20-year-old male patient diagnosed with both anti-GBM disease and TBMN upon presenting dyspnea and hemoptysis.

Patient Concerns: No laboratory abnormalities, except arterial hypoxemia (PaO275. Read More

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Baseline Characteristics and Survival of an Australian Interstitial Pneumonia with Autoimmune Features Cohort.

Respiration 2021 Apr 19:1-12. Epub 2021 Apr 19.

Department of Respiratory, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia.

Background And Objective: The research term "interstitial pneumonia with autoimmune features" (IPAF) encompasses interstitial lung disease (ILD) patients with autoimmune features not meeting diagnostic criteria for a defined connective tissue disease (CTD). It remains unclear if IPAF is a distinct disease entity with implications for management and prognosis. We describe an Australian IPAF population and compare their baseline characteristics and outcomes with distinct cohorts of idiopathic interstitial pneumonia (IIP), CTD-ILD, and unclassifiable ILD. Read More

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Assessment score for the diagnosis of a case with pleuroparenchymal fibroelastosis.

Monaldi Arch Chest Dis 2021 Apr 6. Epub 2021 Apr 6.

Department of Pulmonary Diseases, Cerrahpasa Medical Faculty, Istanbul Cerrahpasa University, Istanbul.

Idiopathic pleuropulmonary fibroelastosis is an extremely rare lung disease characterized by the combination of fibrosis of the visceral pleura and the fibroelastotic changes transcending in the subpleural lung parenchyma that predominantly affects the upper lobes with accompanying volume loss. It is mostly idiopathic while infection, autoimmunity, bone marrow or lung transplantation and genetic predisposition may be associated with the development of PPFE. The disease is exceptionally rare as approximately ninety cases have been reported in the literature currently. Read More

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Pirfenidone in patients with progressive fibrotic interstitial lung diseases other than idiopathic pulmonary fibrosis (RELIEF): a double-blind, randomised, placebo-controlled, phase 2b trial.

Lancet Respir Med 2021 05 30;9(5):476-486. Epub 2021 Mar 30.

Center for Interstitial and Rare Lung Diseases, Justus-Liebig University Giessen, Giessen, Germany; Member of the German Center for Lung Research and Cardiopulmonary Institute, and Agaplesion Lung Clinic Waldhof-Elgershausen, Greifenstein, Germany.

Background: Pirfenidone has been shown to slow disease progression in patients with idiopathic pulmonary fibrosis (IPF). However, there are few treatment options for progressive fibrotic interstitial lung diseases (ILDs)) other than IPF. In view of the pathomechanistic and clinical similarities between IPF and other progressive fibrotic ILDs, we aimed to assess the efficacy and safety of pirfenidone in patients with four non-IPF progressive fibrotic ILDs. Read More

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Surface-bound matrix metalloproteinase-8 on macrophages: Contributions to macrophage pericellular proteolysis and migration through tissue barriers.

Physiol Rep 2021 Mar;9(5):e14778

Division of Pulmonary and Critical Care Medicine, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA.

Objective: MMP-8 binds to surface-bound tissue inhibitor of metalloproteinase-1 (TIMP-1) on PMNs to promote pericellular proteolysis during the development of inflammatory diseases associated with tissue destruction. Little is known about the biology of MMP-8 in macrophages. We tested the hypotheses that: (1) MMP-8 and TIMP-1 are also expressed on the surface of activated macrophages, (2) surface-bound MMP-8 on macrophages promotes TIMP-resistant pericellular proteolysis and macrophage migration through tissue barriers, and (3) MMP-8 binds to surface-bound TIMP-1 on macrophages. Read More

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Identification of Autoimmunity to Peptides of Collagen V α1 Chain as Newly Biomarkers of Early Stage of Systemic Sclerosis.

Front Immunol 2020 12;11:604602. Epub 2021 Feb 12.

Rheumatology Division of the Hospital das Clinicas FMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, Brazil.

Patients with Systemic sclerosis (SSc) presents immune dysregulation, vasculopathy, and fibrosis of the skin and various internal organs. Pulmonary fibrosis leads to SSc-associated interstitial lung disease (ILD), which is the main cause of morbidity and mortality in SSc. Recently autoimmunity to type V collagen (Col V) has been characterized in idiopathic pulmonary fibrosis and show promise to be related to the development in SSc. Read More

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February 2021

Generalized granuloma annulare associated with interstitial lung disease: Good response to doxycycline.

Dermatol Ther 2021 03 17;34(2):e14864. Epub 2021 Feb 17.

American University of Beirut Medical Center, Beirut, Lebanon.

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[Interstitial lung disease associated with connective tissue disease].

Pathologe 2021 Feb 8;42(1):4-10. Epub 2021 Jan 8.

Klinik für Rheumatologie, Klinische Immunologie und Nephrologie, Helios Dr. Horst Schmidt-Kliniken Wiesbaden, Wiesbaden, Deutschland.

Interstitial lung disease (ILD) is the most frequent organ manifestation in rheumatic autoimmune disease. Depending on the underlying autoimmune disease, differently pronounced affections of small airways, interstitial parenchyma, and vessels are found. The group of rheumatic autoimmune diseases mainly includes connective tissue diseases (CTDs), also known as collagen vascular diseases, such as rheumatoid arthritis (RA), systemic sclerosis, (SSc), systemic lupus erythematosus, primary Sjögren's syndrome, idiopathic inflammatory myositis (IIM), and interstitial pneumonia with autoimmune features (IPAF). Read More

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February 2021

[Pulmonary manifestations in adult rheumatic diseases].

S Ewig M Bollow

Z Rheumatol 2021 Feb 4;80(Suppl 1):13-32. Epub 2021 Jan 4.

Klinik für diagnostische und interventionelle Radiologie und Nuklearmedizin, Augusta-Kranken-Anstalt Bochum, Bergstr. 26, 44791, Bochum, Deutschland.

Background: Rheumatic diseases frequently present with pulmonary involvement. All anatomic structures of the lungs can be affected. Interstitial lung diseases are characterized by a system of patterns evident in high-resolution computed tomography (HR-CT) scanning of the lungs. Read More

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February 2021

Influenza H1N1 virus-associated pneumonia often resembles rapidly progressive interstitial lung disease seen in collagen vascular diseases and COVID-19 pneumonia; CT-pathologic correlation in 24 patients.

Eur J Radiol Open 2020 28;7:100297. Epub 2020 Nov 28.

Department of Laboratory of Pathology, Nagasaki University Hospital, Nagasaki, Japan.

Purpose: To describe computed tomography (CT) findings of influenza H1N1 virus-associated pneumonia (IH1N1VAP), and to correlate CT findings to pathological ones.

Methods: The study included 24 patients with IH1N1VAP. Two observers independently evaluated the presence, distribution, and extent of CT findings. Read More

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November 2020

Diagnosis and management of connective tissue disease-associated interstitial lung disease in Australia and New Zealand: A position statement from the Thoracic Society of Australia and New Zealand.

Respirology 2021 01 24;26(1):23-51. Epub 2020 Nov 24.

Department of Respiratory Medicine, Princess Alexandra Hospital, Brisbane, QLD, Australia.

Pulmonary complications in CTD are common and can involve the interstitium, airways, pleura and pulmonary vasculature. ILD can occur in all CTD (CTD-ILD), and may vary from limited, non-progressive lung involvement, to fulminant, life-threatening disease. Given the potential for major adverse outcomes in CTD-ILD, accurate diagnosis, assessment and careful consideration of therapeutic intervention are a priority. Read More

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January 2021

Prognosis after acute exacerbation in patients with interstitial lung disease other than idiopathic pulmonary fibrosis.

Clin Respir J 2021 Mar 10;15(3):336-344. Epub 2020 Dec 10.

Department of Pulmonary Medicine, Seirei Hamamatsu General Hospital, Hamamatsu, Japan.

Background: Acute exacerbation (AE) is recognized as a life-threatening condition with acute respiratory worsening in idiopathic pulmonary fibrosis (IPF). AE also occurs in fibrotic interstitial lung disease (ILD) other than IPF, including other types of idiopathic interstitial pneumonias (IIPs), ILD associated with collagen vascular disease (CVD-ILD), and chronic hypersensitivity pneumonia (CHP). However, the clinical impact after AE in those patients is still unclear. Read More

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Profiling and targeting connective tissue remodeling in autoimmunity - A novel paradigm for diagnosing and treating chronic diseases.

Autoimmun Rev 2021 Jan 12;20(1):102706. Epub 2020 Nov 12.

Institute of Translational Immunology and Research Center for Immune Therapy, University Medical Center, Mainz, Germany; Division of Gastroenterology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, USA. Electronic address:

Connective tissue (ConT) remodeling is an essential process in tissue regeneration, where a balanced replacement of old tissue by new tissue occurs. This balance is disturbed in chronic diseases, often autoimmune diseases, usually resulting in the buld up of fibrosis and a gradual loss of organ function. During progression of liver, lung, skin, heart, joint, skeletal and kidney diseasesboth ConT formation and degradation are elevated, which is tightly linked to immune cell activation and a loss of specific cell types and extracellular matrix (ECM) structures that are required for normal organ function. Read More

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January 2021

CXCL9, CXCL10, and CXCL11; biomarkers of pulmonary inflammation associated with autoimmunity in patients with collagen vascular diseases-associated interstitial lung disease and interstitial pneumonia with autoimmune features.

PLoS One 2020 2;15(11):e0241719. Epub 2020 Nov 2.

Department of Respiratory Medicine and Allergology, Sapporo Medical University School of Medicine, Sapporo, Hokkaido, Japan.

Introduction: Interstitial lung disease (ILD) is a heterogeneous group of diseases characterized by varying degrees of lung inflammation and/or fibrosis. We investigated biomarkers to infer whether patients with collagen vascular diseases associated ILD (CVD-ILD) and interstitial pneumonia with autoimmune features (IPAF) benefit from immunosuppressive therapy.

Materials And Methods: We retrospectively investigated patients with CVD-ILD, IPAF, and idiopathic pulmonary fibrosis (IPF) between June 2013 and May 2017 at our department. Read More

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December 2020

Clinical, Serological, and Histopathological Similarities Between Severe COVID-19 and Acute Exacerbation of Connective Tissue Disease-Associated Interstitial Lung Disease (CTD-ILD).

Front Immunol 2020 2;11:587517. Epub 2020 Oct 2.

Institute of Pathology and Molecular Pathology/Study Center of the German Registry of COVID-19 Autopsies (DeRegCOVID), Bundeswehrkrankenhaus Ulm, Ulm, Germany.

Background And Objectives: Understanding the pathophysiology of respiratory failure in coronavirus disease 2019 (COVID-19) is indispensable for development of therapeutic strategies. Since we observed similarities between COVID-19 and interstitial lung disease in connective tissue disease (CTD-ILD), we investigated features of autoimmunity in SARS-CoV-2-associated respiratory failure.

Methods: We prospectively enrolled 22 patients with RT-PCR-confirmed SARS-CoV-2 infection and 10 patients with non-COVID-19-associated pneumonia. Read More

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November 2020

FOXL1 Regulates Lung Fibroblast Function via Multiple Mechanisms.

Am J Respir Cell Mol Biol 2020 12;63(6):831-842

Department of Respiratory Medicine, Graduate School of Medicine, and.

Fibroblasts provide a structural framework for multiple organs and are essential for wound repair and fibrotic processes. Here, we demonstrate functional roles of FOXL1 (forkhead box L1), a transcription factor that characterizes the pulmonary origin of lung fibroblasts. We detected high transcripts associated with DNA hypomethylation and super-enhancer formation in lung fibroblasts, which is in contrast with fibroblasts derived from other organs. Read More

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December 2020

Detection of Myocardial Fibrosis and Left Ventricular Dysfunction with Cardiac MRI in a Hypertensive Swine Model.

Radiol Cardiothorac Imaging 2020 Aug 27;2(4):e190214. Epub 2020 Aug 27.

Departments of Magnetic Resonance Imaging (B.Z., C.C., J.H., S.Z., M.L.), Animal Experimental Center (X.W., G.Y.), and Pathology (X.D., H.W.), Fuwai Hospital, State Key Laboratory of Cardiovascular Disease, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beilishi Road 167, Xicheng District, Beijing 100037, China; National Heart, Lung and Blood Institute (NHLBI), National Institutes of Health (NIH), Bethesda, Md (A.S., A.A., M.L.); and Key Laboratory of Cardiovascular Imaging (Cultivation), Chinese Academy of Medical Sciences, Beijing, China (M.L., C.C.).

Purpose: To quantitatively evaluate the dynamic changes of extracellular volume (ECV) and native T1 in hypertensive swine over time using histologic findings as standard of reference.

Materials And Methods: Eighteen hypertensive (hypertension group) and six healthy (control group) swine aged 6-12 months were studied. Both groups underwent cardiac MRI, including pre- and postcontrast T1 mapping and late gadolinium enhancement (LGE) imaging at three time points: baseline, 1 month, and 3 months after hypertensive model induction. Read More

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Clinical impact of the radiological indeterminate for usual interstitial pneumonia pattern on the diagnosis of idiopathic pulmonary fibrosis.

Respir Investig 2021 Jan 28;59(1):81-89. Epub 2020 Aug 28.

Department of Respiratory Medicine, Japanese Red Cross Medical Center, 4-1-22 Hiroo Shibuya-ku, Tokyo, 150-8935, Japan. Electronic address:

Background: Idiopathic pulmonary fibrosis (IPF) is a fatal lung disease associated with significant morbidity and mortality. The international clinical practice guidelines for the diagnosis of IPF have recently been revised.

Methods: In this single-center retrospective study conducted between June 2006 and March 2018, 27 patients with a newly classified indeterminate for usual interstitial pneumonia (UIP) pattern on high-resolution computed tomography (HRCT) who had undergone surgical lung biopsy were enrolled at the Japanese Red Cross Medical Center. Read More

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January 2021

Mycophenolate Mofetil Improves Exercise Tolerance in Systemic Sclerosis Patients with Interstitial Lung Disease: A Pilot Study.

Rheumatol Ther 2020 Dec 29;7(4):1037-1044. Epub 2020 Aug 29.

Department of Translational and Precision Medicine, Sapienza University, Rome, Italy.

Introduction: Systemic sclerosis (SSc) is an autoimmune disease characterized by the overproduction of collagen leading to fibrosis of the skin and internal organs. Interstitial lung disease (ILD) is one of the major causes of death in patients with SSc. Exercise tolerance can be investigated by cardio-pulmonary exercise testing (CPET). Read More

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December 2020

High levels of anti-SSA/Ro antibodies in COVID-19 patients with severe respiratory failure: a case-based review : High levels of anti-SSA/Ro antibodies in COVID-19.

Clin Rheumatol 2020 Nov 25;39(11):3171-3175. Epub 2020 Aug 25.

Department of Respiratory Medicine, Japanese Red Cross Kyoto Daiichi Hospital, 15-749 Honmachi, Higashiyama, Kyoto, Japan.

We treated two patients with severe respiratory failure due to coronavirus disease 2019 (COVID-19). Case 1 was a 73-year-old woman, and Case 2 was a 65-year-old-man. Neither of them had a history of autoimmune disease. Read More

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November 2020

Complexities of the glomerular basement membrane.

Nat Rev Nephrol 2021 02 24;17(2):112-127. Epub 2020 Aug 24.

Wellcome Centre for Cell-Matrix Research, Division of Cell-Matrix Biology and Regenerative Medicine, School of Biological Sciences, Faculty of Biology Medicine and Health, The University of Manchester, Manchester Academic Health Science Centre, Manchester, UK.

The glomerular basement membrane (GBM) is a key component of the glomerular capillary wall and is essential for kidney filtration. The major components of the GBM include laminins, type IV collagen, nidogens and heparan sulfate proteoglycans. In addition, the GBM harbours a number of other structural and regulatory components and provides a reservoir for growth factors. Read More

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February 2021

Chronic Hypersensitivity Pneumonitis, an Interstitial Lung Disease with Distinct Molecular Signatures.

Am J Respir Crit Care Med 2020 11;202(10):1430-1444

Department of Medicine.

Chronic hypersensitivity pneumonitis (CHP) is caused by an immune response to antigen inhalation and is characterized by variable histopathological and clinical features. A subset of subjects with CHP have usual interstitial pneumonia and appear to be clinically similar to subjects with idiopathic pulmonary fibrosis (IPF). To determine the common and unique molecular features of CHP and IPF. Read More

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November 2020

Genetic Variation in CCL18 Gene Influences CCL18 Expression and Correlates with Survival in Idiopathic Pulmonary Fibrosis: Part A.

J Clin Med 2020 Jun 21;9(6). Epub 2020 Jun 21.

Centre for Interstitial Lung Diseases, Department of Pulmonology, St. Antonius Hospital, 3435 CM Nieuwegein, The Netherlands.

Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic disease, characterized by fibroblast proliferation and extracellular matrix deposition. CC-chemokine ligand 18 (CCL18) upregulates the production of collagen by lung fibroblasts and is associated with mortality. This study was designed to evaluate the influence of single nucleotide polymorphisms (SNPs) in the gene on CCL18 expression and survival in IPF. Read More

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Experimental Antiglomerular Basement Membrane GN Induced by a Peptide from .

J Am Soc Nephrol 2020 06;31(6):1282-1295

Renal Division, Peking University First Hospital, Beijing, PR China.

Background: Antiglomerular basement membrane (anti-GBM) disease is associated with HLA-DRB1*1501 (the major predisposing genetic factor in the disease), with 3 as a nephritogenic T and B cell epitope. Although the cause of disease remains unclear, the association of infections with anti-GBM disease has been long suspected.

Methods: To investigate whether microbes might activate autoreactive T and B lymphocytes molecular mimicry in anti-GBM disease, we used bioinformatic tools, including BLAST, SYFPEITHI, and ABCpred, for peptide searching and epitope prediction. Read More

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Differential expression of caveolin-1 during pathogenesis of combined pulmonary fibrosis and emphysema: Effect of phosphodiesterase-5 inhibitor.

Biochim Biophys Acta Mol Basis Dis 2020 08 18;1866(8):165802. Epub 2020 Apr 18.

Department of Physiology, Vallabhbhai Patel Chest Institute, University of Delhi, Delhi, India.

Introduction: Combined pulmonary fibrosis and emphysema (CPFE) is a relatively new entity within the spectrum of cigarette smoke induced lung disorders. Currently there is no consensus about its treatment. We hypothesized that caveolin-1 critically determines the parenchymal and vascular remodeling leading to the development of CPFE. Read More

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Idiopathic Pulmonary Hemosiderosis as a Mimic of Pulmonary Vasculitis: A Case Report and Review of the Literature.

Curr Allergy Asthma Rep 2020 04 4;20(5):13. Epub 2020 Apr 4.

Duke Pediatric Rheumatology, Box 3212 2301 Erwin Rd., Durham, NC, 27705, USA.

Purpose Of Review: Idiopathic pulmonary hemosiderosis (IPH) is one of the rarest and least understood causes of pulmonary hemorrhage in children. Illustrated by a complex case presentation, we discuss the clinical manifestations, diagnosis, pathology, proposed etiologies, and treatment of this rare disease. We also compare IPH with anti-glomerular basement membrane antibody syndrome (anti-GBM disease), another rare causes of pediatric pulmonary hemorrhage. Read More

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Clinical course of Japanese patients with early systemic sclerosis: A multicenter, prospective, observational study.

Mod Rheumatol 2021 Jan 21;31(1):162-170. Epub 2020 Apr 21.

Department of Dermatology and Plastic Surgery, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan.

Objectives: To investigate the clinical course of Japanese patients with early diffuse cutaneous systemic sclerosis (dcSSc) and early SSc with interstitial lung disease (ILD).

Methods: We prospectively analyzed the clinical features of 207 Japanese patients with early dcSSc (n = 150) and limited cutaneous SSc (lcSSc) with ILD (n = 57) in 10 medical centers every year for 7 consecutive years.

Results: Mean modified Rodnan total skin thickness score (mRSS) was 18. Read More

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January 2021

Mitochondria dysfunction and metabolic reprogramming as drivers of idiopathic pulmonary fibrosis.

Redox Biol 2020 06 19;33:101509. Epub 2020 Mar 19.

Aging Institute, Department of Medicine, University of Pittsburgh, Pittsburgh, PA, USA; Division of Pulmonary Allergy and Critical Care Medicine, Department of Medicine, University of Pittsburgh, Pittsburgh, PA, USA; Vascular Medicine Institute, Department of Medicine, University of Pittsburgh, Pittsburgh, PA, USA. Electronic address:

Idiopathic pulmonary fibrosis (IPF) is a devastating lung disease of unknown etiology. It is characterized by deposition of extracellular matrix proteins, like collagen and fibronectin in the lung interstitium leading to respiratory failure. Our understanding of the pathobiology underlying IPF is still incomplete; however, it is accepted that aging is a major risk factor in the disease while growing evidence suggests that the mitochondria plays an important role in the initiation and progression of pulmonary fibrosis. Read More

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Thrombospondin-1 mediates muscle damage in brachio-cervical inflammatory myopathy and systemic sclerosis.

Neurol Neuroimmunol Neuroinflamm 2020 05 6;7(3). Epub 2020 Mar 6.

From the Neuromuscular Diseases Unit (X.S.-C., J.A.-P., A.C.-R., E.F.-S., A.A.-J., R.R.-G., J.T., L.Q., N.d.L., E.C.-V., I.I., E.G., J.D.-M.), Neurology Department, Hospital de la Santa CreuiSant Pau and Biomedical Research Institute Sant Pau (IIB Sant Pau), Barcelona; Centro de Investigaciones Biomédicas en Red en Enfermedades Raras (CIBERER) (X.S.-C., R.R.-G., L.Q., N.d.L., E.C.-V., I.I., E.G., J.D.-M.), Madrid; John Walton Muscular Dystrophy Research Center (J.D.-M), University of Newcastle, UK; Rheumatology Unit (I.C., A.M.-n.-M., H.C.), Hospital de la Santa Creu i Sant Pau; Laboratory of Experimental Immunology (C.Z.), Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau (IIB Sant Pau); Servei Immunologia (L.M.-M.), Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau (IIB Sant Pau); and Department of Respiratory Medicine (D.C.), Hospital de la Santa Creu i Sant Pau, Barcelona, Spain.

Objective: To describe the clinical, serologic and histologic features of a cohort of patients with brachio-cervical inflammatory myopathy (BCIM) associated with systemic sclerosis (SSc) and unravel disease-specific pathophysiologic mechanisms occurring in these patients.

Methods: We reviewed clinical, immunologic, muscle MRI, nailfold videocapillaroscopy, muscle biopsy, and response to treatment data from 8 patients with BCIM-SSc. We compared cytokine profiles between patients with BCIM-SSc and SSc without muscle involvement and controls. Read More

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