Microvascular alterations of the ocular surface and retina in connective tissue disease-related interstitial lung disease

AIM:To examine the disparities in macular retinal vascular density between individuals with connective tissue disease-related interstitial lung disease(CTD-ILD)and healthy controls(HCs)by optical coherence tomography angiography(OCTA)and to investigate the changes in microvascular density in abnormal eyes.METHODS:For a retrospective case-control study,a total of 16 patients(32 eyes)diagnosed with CTD-ILD were selected as the ILD group.The 16 healthy volunteers with 32 eyes,matched in terms of age and sex with the patients,were recruited as control group.The macular retina’s superficial retinal layer(SRL)and deep retinal layer(DRL)were examined and scanned using OCTA in each individual eye.The densities of retinal microvascular(MIR),macrovascular(MAR),and total microvascular(TMI)were calculated and compared.Changes in retinal vascular density in the macular region were analyzed using three different segmentation methods:central annuli segmentation method(C1-C6),hemispheric segmentation method[uperior right(SR),superior left(SL),inferior left(IL),and inferior right(IR)],and Early Treatment Diabetic Retinopathy Study(ETDRS)methods[superior(S),inferior(I),left(L),and right(R)].The data were analyzed using Version 9.0 of GraphPad prism and Pearson analysis.RESULTS:The OCTA data demonstrated a statistically significant difference(P<0.05)in macular retinal microvessel density between the two groups.Specifically,in the SRL and DRL analyses,the ILD group exhibited significantly lower surface density of MIR and TMI compared to the HCs group(P<0.05).Furthermore,using the hemispheric segmentation method,the ILD group showed notable reductions in SL,SR,and IL in the superficial retina(P<0.05),as well as marked decreases in SL and IR in the deep retina(P<0.05).Similarly,when employing the ETDRS method,the ILD group displayed substantial drops in superficial retinal S and I(P<0.05),along with notable reductions in deep retinal L,I,and R(P<0.05).In the central annuli segmentation method,the ILD group exhibited a significant decrease in the superficial retinal C2-4 region(P<0.05),whereas the deep retina showed a notable reduction in the C3-5 region(P<0.05).Additionally,there was an observed higher positive likelihood ratio in the superficial SR region and deep MIR.Furthermore,there was a negative correlation between conjunctival vascular density and both deep and superficial retinal TMI(P<0.001).CONCLUSION:Patients with CTD-ILD exhibits a significantly higher conjunctival vascular density compared to the HCs group.Conversely,their fundus retinal microvascular density is significantly lower.Furthermore,CTD-ILD patients display notably lower superficial and deep retinal vascular density in comparison to the HCs group.The inverse correlation between conjunctival vascular density and both superficial and deep retinal TMI suggests that detecting subtle changes in ocular microcirculation could potentially serve as an early diagnostic indicator for connective tissue diseases,thereby enhancing disease management.

Primary biliary cholangitis presenting with granulomatous lung disease misdiagnosed as lung cancer:A case report

BACKGROUND There are few cases of pulmonary granulomatous changes secondary to primary biliary cirrhosis(PBC).No case of granulomatous lung disease secondary to PBC misdiagnosed as lung cancer had been reported.CASE SUMMARY A middle-aged woman presented with lung nodules and was misdiagnosed with lung cancer by positron emission tomography/computed tomography.She underwent left lobectomy,and the pathology of the nodules showed granulomatous inflammation,which was then treated with antibiotics.However,a new nodule appeared.Further investigation with lung biopsy and liver serology led to the diagnosis of PBC,and chest computed tomography indicated significant reduction in the pulmonary nodule by treatment with methylprednisolone and ursodeoxycholic acid.CONCLUSION Diagnosis of pulmonary nodules requires integrating various clinical data to avoid unnecessary pulmonary lobectomy.

Clinical evolution of antisynthetase syndrome-associated interstitial lung disease after COVID-19 in a man with Klinefelter syndrome:A case report

BACKGROUND This study presents a case of rapidly developing respiratory failure due to antisynthetase syndrome(AS)following coronavirus disease 2019(COVID-19)in a 33-year-old man diagnosed with Klinefelter syndrome(KS).CASE SUMMARY A 33-year-old man with a diagnosis of KS was admitted to the Department of Pulmonary and Critical Care Medicine of a tertiary hospital in China for fever and shortness of breath 2 wk after the onset of COVID-19.Computed tomography of both lungs revealed diffuse multiple patchy heightened shadows in both lungs,accompanied by signs of partial bronchial inflation.Metagenomic next-generation sequencing of the bronchoalveolar lavage fluid suggested absence of pathogen.A biopsy specimen revealed organizing pneumonia with alveolar septal thickening.Additionally,extensive auto-antibody tests showed strong positivity for anti-SSA,anti-SSB,anti-Jo-1,and anti-Ro-52.Following multidisciplinary discussions,the patient received a final diagnosis of AS,leading to rapidly progressing respiratory failure.CONCLUSION This study underscores the clinical progression of AS-associated interstitial lung disease subsequent to viral infections such as COVID-19 in patients diagnosed with KS.

Systemic juvenile idiopathic arthritis–associated lung disease: A retrospective cohort study

BACKGROUND Lung damage in systemic juvenile arthritis(sJIA)is one of the contemporary topics in pediatric rheumatology.Several previous studies showed the severe course and fatal outcomes in some patients.The information about interstitial lung disease(ILD)in the sJIA is scarce and limited to a total of 100 cases.AIM To describe the features of sJIA patients with ILD in detail.METHODS In the present retrospective cohort study,information about 5 patients less than 18-years-old with sJIA and ILD were included.The diagnosis of sJIA was made according to the current 2004 and new provisional International League of Associations for Rheumatology criteria 2019.ILD was diagnosed with chest computed tomography with the exclusion of other possible reasons for concurrent lung involvement.Macrophage activation syndrome(MAS)was diagnosed with HLH-2004 and 2016 EULAR/ACR/PRINTO Classification Criteria and hScores were calculated during the lung involvement.RESULTS The onset age of sJIA ranged from 1 year to 10 years.The time interval before ILD ranged from 1 mo to 3 years.The disease course was characterized by the prevalence of the systemic features above articular involvement,intensive rash(100%),persistent and very active MAS(hScore range:194-220)with transaminitis(100%),and respiratory symptoms(100%).Only 3 patients(60%)developed a clubbing phenomenon.All patients(100%)had pleural effusion and 4 patients(80%)had pericardial effusion at the disease onset.Two patients(40%)developed pulmonary arterial hypertension.Infusion-related reactions to tocilizumab were observed in 3(60%)of the patients.One patient with trisomy 21 had a fatal disease course.Half of the remaining patients had sJIA remission and 2 patients had improvement.Lung disease improved in 3 patients(75%),but 1 of them had initial deterioration of lung involvement.One patient who has not achieved the sJIA remission had the progressed course of ILD.No cases of hyper-eosinophilia were noted.Four patients(80%)received canakinumab and one(20%)tocilizumab at the last follow-up visit.CONCLUSION ILD is a severe life-threatening complication of sJIA that may affect children of different ages with different time intervals since the disease onset.Extensive rash,serositis(especially pleuritis),full-blown MAS with transaminitis,lymphopenia,trisomy 21,eosinophilia,and biologic infusion reaction are the main predictors of ILD.The following studies are needed to find the predictors,pathogenesis,and treatment options,for preventing and treating the ILD in sJIA patients.

Correlation Analysis Between Changes of D-Dimer Level and Rheumatoid Arthritis Complicated with Interstitial Lung Disease

Objective:To explore the correlation between the change of D-dimer level and rheumatoid arthritis complicated with interstitial lung disease.Methods:From January 2022 to February 2024,20 rheumatoid arthritis patients complicated with interstitial lung disease(interstitial lung disease group),20 rheumatoid arthritis patients without interstitial lung disease(without interstitial lung disease group),and 20 healthy people(control group)in Xijing Hospital were selected for this study.The fasting venous blood of the three groups of subjects was collected and their D-dimer,C-reactive protein(CRP),rheumatoid factor(RF),and erythrocyte sedimentation rate(ESR)were detected.Subsequently,the correlation between each index and rheumatoid arthritis complicated with interstitial lung disease was analyzed.Results:The D-dimer level of the interstitial lung disease group was significantly higher than the other two groups(P<0.05).The D-dimer level of the group without interstitial lung disease was significantly higher than the control group(P<0.05).CRP levels in the interstitial lung disease group and the group without interstitial lung disease were significantly higher than those of the control group(P<0.05).The ESR and RF levels of the interstitial lung disease group were significantly higher than the other two groups(P<0.05).The levels of ESR and RF levels of the group without interstitial lung disease were significantly higher than the control group(P<0.05).Conclusion:D-dimer levels of rheumatoid arthritis patients are higher than those of healthy individuals,and those complicated with interstitial lung disease present even higher levels.This finding shows that there is a correlation between D-dimer levels and rheumatoid arthritis with interstitial lung disease,which may facilitate the evaluation and diagnosis of this disease.

Effect of N-Acetylcysteine Combined with Lung Rehabilitation Therapy on Exercise Endurance and Quality of Life of Patients with Rheumatoid Arthritis-Related Interstitial Lung Disease

Objective:To explore the effect of N-acetylcysteine combined with lung rehabilitation therapy on exercise endurance and quality of life in patients with rheumatoid arthritis-related interstitial lung disease(RA-ILD).Methods:Fifty-six patients with RA-ILD admitted to Xijing Hospital from May 2022 to January 2024 were randomly divided into two groups:a non-rehabilitation group and a pulmonary rehabilitation group,with 28 patients in each group.Both groups received routine treatment.Additionally,the non-rehabilitation group received N-acetylcysteine treatment,while the lung rehabilitation group received lung rehabilitation treatment in addition to N-acetylcysteine.The improvement in exercise endurance and dyspnea between the two groups after treatment was compared and the quality of life of the patients was observed.Results:After treatment,the exercise endurance score in the lung rehabilitation group(335.67±45.29)was higher than that in the non-rehabilitation group(P<0.05).The dyspnea score in the lung rehabilitation group(0.72±0.16)was lower than that in the non-rehabilitation group(P<0.05).Additionally,FVC(3.18±0.58 L),FEV1(2.28±0.56 L),FEV1/FVC(69.69±5.56),and DLCO(60.53±5.92 mL/mmHg/min)were higher in the lung rehabilitation group compared to the non-rehabilitation group after treatment(P<0.05).Conclusion:Lung rehabilitation therapy combined with N-acetylcysteine treatment can effectively improve dyspnea symptoms,lung function,and exercise endurance in patients with RA-ILD.This approach helps to improve patient’s quality of life and is beneficial for their prognosis.

Nodular Pulmonary Amyloidosis with Interstitial Lung Disease—Case Report and Literature Review

Amyloidosis is a rare spectrum of disease which involves deposition of misfolded extracellular proteins (amyloids) in various body organs leading to progressive organ dysfunction. Clinical presentation can be variable depending on the organ involved and type of protein. Amyloidosis can be classified based on quantity, type, and location of these proteins. Amyloid light-chain amyloidosis develops in the bone marrow, producing abnormal forms of light-chain proteins, which cannot be broken down. These proteins transform into amyloid fibrils and form amyloid deposits in different organs. Pulmonary amyloidosis is uncommonly diagnosed since it is rarely symptomatic. Diagnosis of pulmonary amyloidosis is usually made in the setting of systemic amyloidosis;however, it may present as localised pulmonary disease. Localized pulmonary Amyloidosis can present as nodular, cystic, or tracheobronchial amyloidosis. Depending on the degree of the interstitial involvement, it may affect alveolar gas exchange and cause respiratory symptoms. This is a case of a 47-year-old female with background history of interstitial lung disease presenting with progressive shortness of breath. Computed tomography scan revealed bilateral pulmonary nodules. The patient was referred to our thoracic surgery team with the suspicion of bronchogenic malignancy with metastasis. Diagnostic video assisted wedge resection was performed for this patient, and histology confirmed pulmonary amyloidosis of nodular type. Amyloid deposition simulates both inflammatory and neoplastic conditions. Definitive diagnosis requires biopsy confirmation therefore early detection and commencing the patient on appropriate treatment pathway may help in symptomatic relief and better outcome.

Acute exacerbation of interstitial lung disease in the intensive care unit:Principles of diagnostic evaluation and management

Interstitial lung disease(ILD)is typically managed on an outpatient basis.Critical care physicians manage patients with ILD in the setting of an acute exacerbation(ILD flare)causing severe hypoxia.The principles of management of acute exacerbation of ILD are different from those used to manage patients with acute respiratory distress syndrome from sepsis,etc.Selected patients may be candidates for aggressive measures like extracorporeal membrane oxygenation and lung transplantation,while almost all patients will benefit from early palliative care.This review focused on the types of ILD,diagnosis,and management pathways for this challenging condition.

Mechanism prediction of Astragalus mongholicus Bunge and Angelica sinensis Diels in treating interstitial lung disease based on network pharmacology and molecular docking

Objective:To investigate the mechanism by which Astragalus mongholicus Bunge(AM),and Angelica sinensis Diels(AS)act in interstitial lung disease(ILD)based on computational prediction.Methods:We screened the ingredients of AM and AS in PubMed,the Web of Science,China National Knowledge Infrastructure(CNKI)Databases,etc.Then obtained the potential effective components.By sharing the same molecular with ILD,we got the possible target genes for ILD treatment and constructed components–targets–disease network with Cytoscape software.The CTD(Comparative Toxicogenomics Database)database was used for GO and KEGG enrichment analysis of these target genes.Results:59 active ingredients that can be druggable were chosen from AM,67 active ingredients were chosen from AS.77 overlapping target genes for AM and ILD and 36 overlapping target genes for AS and ILD were acquired.The hub targets of AM were PTGS2,PTGS1,CDK2,MAOA,ESR1,TOP2A,GSK3B,ESR2,PPARG,NOS2,The hub targets of AS were PTGS2,GABRA1,PTGS1,CHRM1,SLC6A2,ADRA1B,ADRAIA,ADRB2,CHRM3,GABRA2,CHRM2.Quercetin,kaempferol,daidzein,pavilion,7-Hydroxycoumarin,and 5-Hydroxycoumarin were the main active ingredients which have more effective targets.Prediction of the protein-protein interaction network showed PTGS2,GSK3B,PPARG,etc.,were the important predicted targets.The enriched KEGG pathways,including the Immune System,Metabolism of lipids and lipoproteins,Cytokine Signaling in the Immune system,Generic Transcription Pathway,The interleukin pathway,Metabolism of proteins,PI3K-Akt signaling pathway,Metabolic pathways,Innate Immune System,Neuroactive ligand-receptor interaction,Metabolism,GPCR downstream signaling,Amine ligand-binding receptors,Class A/1,Calcium signaling pathway.Molecular docking showed that quercetin,kaempferol,daidzein,pavilion,7-Hydroxycoumarin,5-Hydroxycoumarin had good binding activities with PTGS2 and GSK3B,which mainly mediated PI3K/Akt and other important signaling pathways in the pathogenesis of ILD.Conclusion:The components in AS and AM share some common targets,such as PTGS2.AM and AS may ameliorate ILD through the PI3K-Akt signaling pathway which is mediated by GSK3B.PTGS2,PPARG may also be vital target genes in the treatment of ILD with AM and AS.

Expert Consensus on the Diagnosis and Treatment of Anticancer Drug-Induced Interstitial Lung Disease

Drug-induced interstitial lung disease(DILD)is the most common pulmonary adverse event of anticancer drugs.In recent years,the incidence of anticancer DILD has gradually increased with the rapid development of novel anticancer agents.Due to the diverse clinical manifestations and the lack of specific diagnostic criteria,DILD is difficult to diagnose and may even become fatal if not treated properly.Herein,a multidisciplinary group of experts from oncology,respiratory,imaging,pharmacology,pathology,and radiology departments in China has reached the“expert consensus on the diagnosis and treatment of anticancer DILD”after several rounds of a comprehensive investigation.This consensus aims to improve the awareness of clinicians and provide recommendations for the early screening,diagnosis,and treatment of anticancer DILD.This consensus also emphasizes the importance of multidisciplinary collaboration while managing DILD.

Association between the Lung Immune Prognostic Index and mortality in patients with idiopathic inflammatory myopathy-associated interstitial lung disease

Objective:To explore the association between the Lung Immune Prognostic Index(LIPI)and 1-year all-cause mortality in patients with idiopathic inflammatory myopathy related interstitial lung disease(IIM-ILD).Methods:Patients who were diagnosed with IIM-ILD at West China Hospital,Sichuan University from January 2008 to December 2021 were retrospectively included and categorized into three groups based on LIPI.Univariable and multivariable Cox proportional hazards models were conducted to explore potential association between the LIPI and patients'mortality.Results:A total of 1116 patients were screened,and 830 were included in this study.The multivariable Cox analysis showed that,compared with patients with poor LIPI,the hazard ratio(HR)for all-cause 1-year mortality was 0.22(95%CI 0.05-0.93,P=0.04)for patients in the good LIPI group(LDH<250 IU/L and dNLR<3).After excluding patients lost to follow-up within one year,a similar result was found for LIPI(HR 0.20,95%CI 0.05-0.86;P=0.03).Conclusions:Good LIPI was independently associated with decreased risk of all-cause 1-year mortality in patients with IIM-ILD.This easy-to-obtain index might be served as a potential marker for assessing the prognosis of IIM-ILD.

Molecular mechanisms and cellular process in signal transduction pathway related to air pollutants in obstructive lung diseases:A mini-review

Exposure to air pollutants such as PM_(10),PM_(2.5),PM_(0.1),O_(3),CO,NO2,and SO_(2),and biological pollutants are important factors causing the evolution and furtherance of obstructive lung diseases(OLD),including asthma and chronic obstructive pulmonary disease(COPD).Asthma is the most frequent chronic inflammatory airway disease,characterized by breathlessness,wheezing,chest tightness,and cough,together with the presence of exaggerated expiratory airflow fluctuation that varies over time.COPD is a heterogeneous lung condition characterized by chronic respiratory symptoms such as dyspnea,cough,expectoration,and/or exacerbations due to abnormalities of the airways and/or alveoli that cause persistent,often progressive,airflow obstruction.Understanding the molecular mechanisms and cellular processes based on the development of OLD on exposure to air pollutants will provide insights into the solution of pathogenesis,prevention,and treatment of these conditions.The molecular mechanisms and cellular process involved in signal transduction pathway plays a role in the binding of extracellular signaling molecules and ligands to receptors placed on the cell surface or on the inner side cell that trigger inflammation that occurs,especially when something important enters the cell to bring into a cascade response.This binding then alters the cell metabolism,shape,and gene expression in the airway.This review aimed to reveal the effect of air pollutants on the molecular mechanisms and cellular processes involved in the signal transduction pathways in OLD.

Anti-melanoma differentiation-associated gene 5 and anti-Ro52 antibody-dual positive dermatomyositis accompanied by rapidly lung disease:Three case reports

BACKGROUND Clinically amyopathic deramatomyositis was manifested as the various cutaneous dermatomyositis(DM)manifestations without muscle weakness.Anti-melanoma differentiation-associated gene 5(anti-MDA5)and anti-Ro52 antibody-dual positive clinically amyopathic DM patients are at a high risk of developing rapidly progressive interstitial lung disease,and they exhibit an immensely high half-year mortality.CASE SUMMARY We presented three patients with anti-MDA5 and anti-Ro52 antibody-dual positive DM patients and we reviewed the previous studies on the link between anti-MDA5 and anti-Ro52 antibody-dual positive DM.Although we aggressively treated these patients similarly,but they all exhibited different prognoses.We reviewed the importance of clinical cutaneous rashes as well as the pathogenesis and treatment in the dual positive anti-MDA5 and anti-Ro52 associated DM.CONCLUSION Patients with anti-MDA5 anti-Ro52 antibody-dual positive DM should be accurately diagnosed at an early stage and should be treated aggressively,thus,the patient’s prognosis can be significantly modified.

A Validated Model for the Imaging Diagnosis of Cystic Lung Disease

Rationale and Objectives: Cystic lung disease may be accurately diagnosed by imaging interpretation of specialist radiologists, without other information. We hypothesized that with minimal training non-specialists could perform similarly to specialist physicians in the diagnosis of cystic lung disease. Methods: 72 cystic lung disease cases and 25 cystic lung disease mimics were obtained from three sources: 1) a prospective acquired diffuse lung disease registry, 2) a retrospective search of medical records and 3) teaching files. Cases were anonymized, randomized and interpreted by 7 diffuse lung disease specialists and 15 non-specialist radiologists and pulmonologists. Clinical information other than age and sex was not provided. Prior to interpretation, non-specialists viewed a short PDF training document explaining cystic lung disease interpretation. Results: Correct first choice diagnosis of 85%-88% may be achieved by high-performing specialist readers and 71%-80% by non-specialists and lower-performing specialists, with mean accuracies in the diagnosis of LAM (91%, p Conclusion: With specific but limited training, non-specialist physicians can diagnose cystic lung diseases from CT appearance alone with similar accuracy to specialists, correctly identifying approximately 75% of cases.

Mechanism prediction of Astragalus mongholicus Bunge and Angelica sinensis Diels in treating interstitial lung disease based on network pharmacology and molecular docking

Objective:To investigate the mechanism by which Astragalus mongholicus Bunge(AM),and Angelica sinensis Diels(AS)act in interstitial lung disease(ILD)based on computational prediction.Methods:We screened the ingredients of AM and AS in PubMed,the Web of Science,China National Knowledge Infrastructure(CNKI)Databases,etc.Then obtained the potential effective components.By sharing the same molecular with ILD,we got the possible target genes for ILD treatment and constructed components–targets–disease network with Cytoscape software.The CTD(Comparative Toxicogenomics Database)database was used for GO and KEGG enrichment analysis of these target genes.Results:59 active ingredients that can be druggable were chosen from AM,67 active ingredients were chosen from AS.77 overlapping target genes for AM and ILD and 36 overlapping target genes for AS and ILD were acquired.The hub targets of AM were PTGS2,PTGS1,CDK2,MAOA,ESR1,TOP2A,GSK3B,ESR2,PPARG,NOS2,The hub targets of AS were PTGS2,GABRA1,PTGS1,CHRM1,SLC6A2,ADRA1B,ADRAIA,ADRB2,CHRM3,GABRA2,CHRM2.Quercetin,kaempferol,daidzein,pavilion,7-Hydroxycoumarin,and 5-Hydroxycoumarin were the main active ingredients which have more effective targets.Prediction of the protein-protein interaction network showed PTGS2,GSK3B,PPARG,etc.,were the important predicted targets.The enriched KEGG pathways,including the Immune System,Metabolism of lipids and lipoproteins,Cytokine Signaling in the Immune system,Generic Transcription Pathway,The interleukin pathway,Metabolism of proteins,PI3K-Akt signaling pathway,Metabolic pathways,Innate Immune System,Neuroactive ligand-receptor interaction,Metabolism,GPCR downstream signaling,Amine ligand-binding receptors,Class A/1,Calcium signaling pathway.Molecular docking showed that quercetin,kaempferol,daidzein,pavilion,7-Hydroxycoumarin,5-Hydroxycoumarin had good binding activities with PTGS2 and GSK3B,which mainly mediated PI3K/Akt and other important signaling pathways in the pathogenesis of ILD.Conclusion:The components in AS and AM share some common targets,such as PTGS2.AM and AS may ameliorate ILD through the PI3K-Akt signaling pathway which is mediated by GSK3B.PTGS2,PPARG may also be vital target genes in the treatment of ILD with AM and AS.

Cutaneous Cryptococcosis Arising in a Patient with Idiopathic Lung Disease: Related Illnesses or “Ticks and Fleas”

Cryptococcus is a yeast typically found in bird feces such as pigeon droppings. Infection may occur through inhalation of spores or via direct inoculation into the skin. Typically there is a history of immunosuppression, but cases are also reported in immunocompetent hosts. Cryptococcus may manifest as pulmonary disease or primary cutaneous infection, but dissemination to a systemic illness is the most life-threatening concern. We present the case of a 71-year-old man with a four-year history of idiopathic lung disease, treated with oral prednisone and mycophenolate, presents with a new onset skin rash on the right wrist. He has a history of cleaning and clearing barns and sheds after a recent storm devastated the area. Birds and bats were present in these structures while he was working. Initial therapy failed, and subsequent biopsy showed the presence of Cryptococcus yeast. Further investigation yielded a positive, low titer Cryptococcus antigen screen but negative blood cultures. This case illustrates three valuable facets of patient care. Ideally, one diagnosis will explain all of the clinical presentation, but when that is not the case then multiple etiologies must be explored. Sometimes first-line therapy is ineffective, and the clinician should not be afraid to recognize that and change course. Importantly with skin lesions, failure to respond to treatment or worsening of the lesion in the face of topical and/or oral steroids should lead one to consider the possibility of infection, particularly in an immunosuppressed patient, and prompt biopsy is prudent.

Interstitial lung disease and diabetes

Diabetes mellitus (DM) is a chronic metabolic disease and its prevalence has beensteadily increasing all over the world. DM and its associated micro andmacrovascular complications result in significant morbidity and mortality. Themicrovascular complications are usually manifested as retinopathy, neuropathy,nephropathy and macrovascular complications generally affect the cardiovascularsystem. In addition to these complications, DM also affects the lungs because of itsrich vascularity and abundance in connective tissue (collagen and elastin). DMhas been found to cause microvascular complications and proliferation ofextracellular connective tissue in the lungs, leading to decline in lung function in arestrictive pattern. Interstitial lung disease (ILD) includes a diverse group ofdisease conditions characterized by different degrees of inflammation and fibrosisin the pulmonary parenchyma. Idiopathic pulmonary fibrosis (IPF) is one of thecommon type of idiopathic interstitial pneumonia with a high mortality rate. IPFis characterized by chronic progressive fibrosis leading to progressive respiratoryfailure. In this review we focus on lung as the target organ in DM and theassociation of DM and ILD with special emphasis on IPF.

Reliability and validity of Chinese version of a tool to assess the quality of life in idiopathic pulmonary fibrosis in patients with interstitial lung disease

Objective:This paper aims to determine the reliability and validity of the Chinese version of a tool that assesses the quality of life in idiopathic pulmonary fibrosis(cATAQ-IPF)in patients with interstitial lung disease(ILD).Methods:We used the process of scale introduction to establish cATAQ-IPF.The content validity of the scale was evaluated by six experts.A total of 92 patients with ILD completed the cATAQ-IPF,St.George's respiratory questionnaire(SGRQ),and The Medical Research Council dyspnoea scale at the baseline,and 15 patients completed cATAQ-IPF at the follow-up period 2 weeks later.Thus,yielding data were used to assess various psychometric properties of cATAQ-IPE Intraclass correlation coefficient(ICC),Cronbach'sαcoefficient,content validity index(CVI),item-level CVI(I-CVI),Pearson's coefficients,criterion-relation validity,and known-group validity were used for data analysis.Results:The cATAQ-IPF showed excellent test-retest reliability(ICC=0.95),except for the therapy domain(Cronbach'sα=0.60)and acceptable internal consistency(Cronbach'sα=0.96 for the total).The scale-level CVI was 0.80,and the I-CVI was in the range of 0.78-1.00.The total cATAQ-IPF score was strongly correlated with the SGRQ total score(r=0.71,P<0.01).The cATAQ-IPF score of patients with ILD was 250.74±47.39,and that of patients with IPF was 287.90±22.56.Patients with IPF possessed considerable impairments in health-related quality of life according to the cATAQ-IPF score(t=4.94,P<0.01).Conclusions:The cATAQ-IPF is a reliable and valid instrument for the evaluation of quality of life of Chinese patients with various forms of ILD.

Vedolizumab-associated diffuse interstitial lung disease in patients with ulcerative colitis:A case report

BACKGROUND Vedolizumab,a newer class of integrin antagonist biological agents,has been applied to treat patients with moderate-to-severe Crohn’s disease(CD)and ulcerative colitis(UC),especially for patients who are refractory to traditional therapies and tumor necrosis factor antagonists.However,some rare but lifethreatening adverse effects warrant pharmacovigilance.We describe the first fatal case of vedolizumab-associated severe diffuse interstitial lung disease in China.CASE SUMMARY We present a case of new-onset diffuse parenchymal lung disease developing under treatment with vedolizumab in a patient with UC.After two doses of vedolizumab,he developed persistent fever and progressively worsening dyspnea.Extensive workups,including bronchoalveolar lavage,transbronchial lung biopsy and metagenomic next-generation sequencing,identified no infectious causes,and other potential causes(such as tumors and cardiogenic pulmonary edema)were also excluded.As a result,a diagnosis of vedolizumabrelated interstitial lung disease was established.Unfortunately,although corticosteroids and empiric antibiotics were administered,the patient eventually died of respiratory failure.CONCLUSION Vedolizumab-related interstitial lung disease in patients with UC is rare but potentially lethal.Gastroenterologists and pulmonologists should be aware of vedolizumab-related adverse drug reactions.

Acute exacerbation of interstitial lung disease in the intensive care unit

Acute exacerbations of interstitial lung disease(AE-ILD)represent an acute,frequent and often highly morbid event in the disease course of ILD patients.Admission in the intensive care unit(ICU)is very common and the need for mechanical ventilation arises early.While non-invasive ventilation has shown promise in staving off intubation in selected patients,it is unclear whether mechanical ventilation can alter the exacerbation course unless it is a bridge to lung transplantation.Risk stratification using clinical and radiographic findings,and early palliative care involvement,are important in ICU care.In this review,we discuss many of the pathophysiological aspects of AE-ILD and raise the hypothesis that ventilation strategies used in acute respiratory distress syndrome might be implemented in AE-ILD.We present possible decision-making and management algorithms that can be used by the intensivist when caring for these patients.