RESUMEN
AIM: Pepsin is an enzyme that helps digest protein secreted only from the gastric chief cell in an inactive state. Pepsin is a good marker for acidic gastroesophageal reflux (GER). Its presence in sputum or saliva is considered pathologic. In GER, cough is stimulated by broncho-esophageal neurogenic reflex and aspiration of gastric contents into the airways. GER is the most common cause of cough. Gastric acid reflux is also thought to play a role in Interstitial Lung Disease (ILD) etiology. In many studies, pepsin and bile acid levels in bronchial lavage were high in patients with interstitial lung disease and chronic cough. In our study, we aimed to evaluate pepsin levels in bronchial lavage in patients with ILD and chronic cough and to investigate the relationship between symptoms and reflux treatment. METHODS: Between January 2021 and February 2022, 212 patients who underwent bronchoscopy in our tertiary clinic were evaluated. These patients were divided into three groups: 52 patients with interstitial lung disease, 81 patients with chronic cough, and 79 patients who underwent bronchoscopy with a pre-diagnosis of lung cancer as the control group. Bronchial lavage obtained by bronchoscopy was analyzed for pepsin levels. RESULTS: Shortness of breath and cough were the most common symptoms in all three groups. Pepsin levels were 16.71 ± 8.6 ng/ml in the chronic cough group, 15.6 ± 8.9 ng/ml in the ILD group, and 10.58 ± 5.4 ng/ml in the lung cancer (control) group. Pepsin levels in the ILD and chronic cough group were statistically significantly higher than in the lung cancer group (p:0.00). There was no statistical difference between the ILD group and the chronic cough group regarding pepsin levels. It was found that pepsin levels were lower in the three groups who received anti-reflux treatment. There was no difference in pepsin levels between ILD subgroups. CONCLUSION: Pepsin levels in bronchial lavage were higher in the ILD and chronic cough groups. This suggests that reflux may be involved in the etiology of chronic cough and ILD. Low pepsin values in patients receiving anti-reflux therapy have shown that occult reflux may occur. In our study, the high level of pepsin in bronchial lavage, especially in the chronic cough and ILD group, may be instructive in the etiology and treatment planning of the disease.
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Tos , Reflujo Gastroesofágico , Enfermedades Pulmonares Intersticiales , Pepsina A , Humanos , Tos/metabolismo , Tos/etiología , Pepsina A/análisis , Pepsina A/metabolismo , Enfermedades Pulmonares Intersticiales/metabolismo , Enfermedades Pulmonares Intersticiales/complicaciones , Enfermedad Crónica , Masculino , Femenino , Persona de Mediana Edad , Reflujo Gastroesofágico/complicaciones , Reflujo Gastroesofágico/metabolismo , Anciano , Líquido del Lavado Bronquioalveolar/química , Lavado Broncoalveolar/métodos , Broncoscopía/métodos , Biomarcadores/metabolismo , Biomarcadores/análisis , Tos CrónicaAsunto(s)
Biomarcadores , Quimiocina CXCL16 , Dermatomiositis , Progresión de la Enfermedad , Enfermedades Pulmonares Intersticiales , Humanos , Enfermedades Pulmonares Intersticiales/complicaciones , Dermatomiositis/complicaciones , Dermatomiositis/sangre , Biomarcadores/sangre , Pronóstico , Quimiocina CXCL16/sangre , Femenino , MasculinoAsunto(s)
Biomarcadores , Quimiocina CXCL16 , Dermatomiositis , Progresión de la Enfermedad , Enfermedades Pulmonares Intersticiales , Enfermedades Pulmonares Intersticiales/inmunología , Enfermedades Pulmonares Intersticiales/sangre , Enfermedades Pulmonares Intersticiales/complicaciones , Humanos , Dermatomiositis/sangre , Dermatomiositis/complicaciones , Dermatomiositis/inmunología , Biomarcadores/sangre , Pronóstico , Quimiocina CXCL16/sangreRESUMEN
The role of nutritional status as a prognostic factor in patients with Sjögren's syndrome-associated interstitial lung disease (SjS-ILD) is currently unclear. This study aimed to predict the prognosis of patients with SjS-ILD through their nutritional status assessment. In this retrospective observational study, nutritional status was evaluated at the time of diagnosis using body mass index (BMI) and nutritional markers such as controlling nutritional status (CONUT), the Glasgow prognostic score (GPS), and prognostic nutrition index (PNI) for all participants. Receiver operating characteristic (ROC) analyses were performed using BMI and each nutritional marker data to compare the area under the ROC curve (AUC) and find the cutoff value using the maximum Youden index. Kaplan-Meier analysis and Cox proportional hazards regression analysis were performed to predict the prognosis of SjS-ILD patients. A total of 112 SjS-ILD patients were enrolled in the study, and 8.9% died during the follow-up period. The median time from diagnosis to follow-up period was 4.2 years. The AUC for PNI was the highest among nutritional markers and BMI, and PNI cutoff value was used to distinguish between the PNI < 47.7 and PNI ≥ 47.7 groups. A statistical difference was observed in the Kaplan-Meier analysis and log-rank test (p = 0.005). In multivariable analyses, PNI < 47.7 (hazard ratio 9.40, 95% confidence interval 1.54-57.21) is associated with increased mortality, suggesting the importance of early nutritional intervention for malnutrition in SjS-ILD patients.
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Enfermedades Pulmonares Intersticiales , Desnutrición , Síndrome de Sjögren , Humanos , Femenino , Enfermedades Pulmonares Intersticiales/mortalidad , Enfermedades Pulmonares Intersticiales/complicaciones , Masculino , Síndrome de Sjögren/complicaciones , Síndrome de Sjögren/mortalidad , Persona de Mediana Edad , Desnutrición/complicaciones , Desnutrición/mortalidad , Estudios Retrospectivos , Anciano , Pronóstico , Estado Nutricional , Curva ROC , Índice de Masa Corporal , Estimación de Kaplan-Meier , Evaluación Nutricional , Modelos de Riesgos ProporcionalesRESUMEN
INTRODUCTION: Refractory or unexplained chronic cough (RUCC) is a common clinical problem with no effective diagnostic tools. The Sensations and Triggers Provoking Cough questionnaire (TOPIC) was developed to characterise cough in RUCC versus cough in other conditions. METHODS: Content analysis of participant interviews discussing the sensations and triggers of chronic cough informed TOPIC development. Participants with chronic cough completed the draft-TOPIC (a subset repeating 5-7 days later), St George's Respiratory Questionnaire (SGRQ), Cough Severity Diary (CSD) and Global Rating of Change Scale. The draft-TOPIC item list was reduced in hierarchical and Rasch analysis to refine the questionnaire to the TOPIC. RESULTS: 49 items describing the triggers and sensations of cough were generated from participant interviews (RUCC n=14, chronic obstructive pulmonary disease (COPD) n=11, interstitial lung disease (ILD) n=10, asthma n=11, bronchiectasis n=3, cystic fibrosis n=7). 140 participants (median age 60.0 (19.0-88.0), female 56.4%; RUCC n=39, ILD n=38, asthma n=45, COPD n=6, bronchiectasis n=12) completed draft-TOPIC, where items with poor 'fit' for RUCC were removed to create TOPIC (8 trigger items, 7 sensation items). Median TOPIC score was significantly higher in RUCC (37.0) vs ILD (24.5, p=0.009) and asthma (7.0, p<0.001), but not bronchiectasis (20.0, p=0.318) or COPD (18.5, p=0.238), likely due to small sample sizes. The Rasch model demonstrated excellent fit in RUCC (χ2=22.04, p=0.85; PSI=0.88); as expected. When all participant groups were included, fit was no longer demonstrated (χ2=66.43, p=0.0001, PSI=0.89) due to the increased heterogeneity (CI=0.077). TOPIC correlated positively with SGRQ (r=0.47, p<0.001) and CSD (r=0.63, p<0.001). The test-retest reliability of TOPIC (intraclass correlation coefficient) was excellent (r=0.90, p<0.001). CONCLUSIONS: High TOPIC scores in the RUCC patients suggest their cough is characterised by specific sensations and triggers. Validation of TOPIC in cough clinics may demonstrate value as an aid to identify features of RUCC versus cough in other conditions.
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Tos , Humanos , Tos/etiología , Tos/diagnóstico , Femenino , Masculino , Persona de Mediana Edad , Anciano , Encuestas y Cuestionarios , Enfermedad Crónica , Adulto , Anciano de 80 o más Años , Adulto Joven , Sensación , Enfermedad Pulmonar Obstructiva Crónica/diagnóstico , Enfermedad Pulmonar Obstructiva Crónica/fisiopatología , Enfermedad Pulmonar Obstructiva Crónica/complicaciones , Bronquiectasia/diagnóstico , Bronquiectasia/fisiopatología , Asma/diagnóstico , Asma/complicaciones , Asma/fisiopatología , Índice de Severidad de la Enfermedad , Reproducibilidad de los Resultados , Enfermedades Pulmonares Intersticiales/diagnóstico , Enfermedades Pulmonares Intersticiales/fisiopatología , Enfermedades Pulmonares Intersticiales/complicaciones , Fibrosis Quística/complicaciones , Fibrosis Quística/fisiopatología , Tos CrónicaRESUMEN
BACKGROUND: Progressive pulmonary fibrosis is the symptomatic, physiological, and radiological progression of interstitial lung diseases. The aim of this study was to examine the relationship between progressive pulmonary fibrosis and demographic characteristics and to evaluate the effect on clinical outcomes and mortality. METHODS: This cross-sectional study included 221 patients diagnosed with non-idiopathic pulmonary fibrosis interstitial lung diseases who were followed in the last 5 years. Patient symptoms, clinical, radiological, and demographic data were examined. Risk factors for the development of progressive pulmonary fibrosis and the relationship with clinical outcomes and mortality were examined. RESULTS: Of the patients, 33.0% (n = 73) had fibrotic idiopathic nonspecific interstitial pneumonia (iNSIP), 35.7% (n = 79) had fibrotic hypersensitivity pneumonia (HP), 18.1% (n = 40) had fibrotic connective tissue disease (CTD) interstitial lung diseases (ILD), and 13.1% (n = 29) had postinfectious fibrotic ILD. The progressive pulmonary fibrosis development rates of the subtypes were 46.5% iNSIP (n = 34), 86.0% fibrotic HP (n = 68), 42.5% fibrotic CTD-ILD (n = 17), and 20.7% postinfectious ILD (n = 6). The presence of progressive pulmonary fibrosis was associated with the development of respiratory failure and mortality (odds ratio [OR]: 2.70, 95% CI: 1.04-7.05 and OR: 2.13, 95% CI: 1.23-3.69). Progressive pulmonary fibrosis development was higher in hypersensitivity pneumonia patients with farmer's lung (OR: 5.06, 95% CI: 1.02-25.18). CONCLUSION: Progressive pulmonary fibrosis was more prevalent in older patients. Farming was an important risk factor in the development of hypersensitivity pneumonia-progressive pulmonary fibrosis. Respiratory failure and mortality were higher in those who developed progressive pulmonary fibrosis.
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Progresión de la Enfermedad , Enfermedades Pulmonares Intersticiales , Fibrosis Pulmonar , Humanos , Estudios Transversales , Masculino , Femenino , Persona de Mediana Edad , Anciano , Enfermedades Pulmonares Intersticiales/mortalidad , Enfermedades Pulmonares Intersticiales/complicaciones , Enfermedades Pulmonares Intersticiales/epidemiología , Fibrosis Pulmonar/epidemiología , Fibrosis Pulmonar/complicaciones , Fibrosis Pulmonar/mortalidad , Factores de Riesgo , Alveolitis Alérgica Extrínseca/complicaciones , Alveolitis Alérgica Extrínseca/patología , Alveolitis Alérgica Extrínseca/epidemiología , Fibrosis Pulmonar Idiopática/complicaciones , Fibrosis Pulmonar Idiopática/mortalidad , Fibrosis Pulmonar Idiopática/epidemiología , Fibrosis Pulmonar Idiopática/patología , Adulto , Enfermedades del Tejido Conjuntivo/complicacionesAsunto(s)
Artritis Reumatoide , Biomarcadores , Enfermedades Pulmonares Intersticiales , Humanos , Artritis Reumatoide/complicaciones , Enfermedades Pulmonares Intersticiales/complicaciones , Enfermedades Pulmonares Intersticiales/etiología , Enfermedades Pulmonares Intersticiales/diagnóstico , Biomarcadores/sangre , Biomarcadores de Tumor/sangre , InflamaciónRESUMEN
BACKGROUND: The early detection and management of (progressive) interstitial lung disease in patients with connective tissue diseases requires the attention and skills of a multidisciplinary team. However, there are currently no well-established standards to guide the daily practice of physicians treating this heterogenous group of diseases. RESEARCH QUESTION: This paper aimed to identify gaps in scientific knowledge along the journey of patients with connective tissue disease-related interstitial lung disease and to provide tools for earlier identification of interstitial lung disease and progressive disease. STUDY DESIGN AND METHODS: The opinions of an international expert panel, which consisted of pulmonologists and rheumatologists were collected and interpreted in the light of peer-reviewed data. RESULTS: Interstitial lung disease is a common complication of connective tissue diseases, but prevalence estimates vary by subtype. Screening and monitoring by means of clinical examination, chest radiography, pulmonary function testing, and disease-specific biomarkers provide insight into the disease activity of patients presenting with connective tissue diseases in a routine setting. Multiple phenotypic and genotypic characteristics have been identified as predictors of the development and progression of interstitial lung disease. However, these risk factors differ between subtypes. To ensure earlier diagnosis of rapidly progressive phenotypes, a risk-based method is necessary for determining the need for HRCT and additional testing. INTERPRETATION: To reduce the underdiagnosis of CTD-ILDs in clinical practice, a standardized and systematic multidisciplinary risk-based approach is suggested. Collaboration across disciplines is essential for the management of CTD-ILD.
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Enfermedades del Tejido Conjuntivo , Diagnóstico Precoz , Enfermedades Pulmonares Intersticiales , Enfermedades Pulmonares Intersticiales/diagnóstico , Enfermedades Pulmonares Intersticiales/etiología , Enfermedades Pulmonares Intersticiales/complicaciones , Humanos , Enfermedades del Tejido Conjuntivo/diagnóstico , Enfermedades del Tejido Conjuntivo/complicaciones , Progresión de la Enfermedad , Guías de Práctica Clínica como Asunto , BiomarcadoresRESUMEN
BACKGROUND: Pulmonary arterial hypertension (PAH)-specific therapies are generally ineffective in patients with pulmonary hypertension associated with lung disease (PH-LD). The aim of this preliminary study was to evaluate the potential efficacy of selexipag, titrated according to individual tolerance, in patients with PH-LD. METHODS: Consecutive patients diagnosed with PH-LD between October 2016 and March 2019, who received selexipag treatment, were retrospectively evaluated. Specific parameters, including changes in hemodynamic parameters, 6-min walk distance (6MWD), and partial pressure of atrial oxygen/fraction of inspiratory oxygen (PaO2/FiO2) were evaluated. Patients whose 6MWD improved ≥20 m were defined as responders. RESULTS: Eight patients with PH-LD were included, comprising four with chronic obstructive pulmonary disease (COPD), two with interstitial lung disease (ILD) related to rheumatoid arthritis, one with ILD related to systemic sclerosis, and one with pulmonary Langerhans cell histiocytosis. No statistically significant improvements in hemodynamic parameters and 6MWD were noted following selexipag treatment. However, four patients showed improvements in 6MWD ≥20 m at follow-up and were considered responders. They had a higher body mass index (BMI) and lower PaO2/FiO2 at baseline than non-responders (p = 0.02 and p = 0.04, respectively). No Grade 3 or 4 adverse events were observed. CONCLUSIONS: Selexipag was effective in half of the PH-LD cases, emphasizing higher BMI and lower PaO2/FiO2 as possible indicators for favorable response. Since selexipag starting at a low dose with subsequent titration may reduce the risk of early adverse events, it can be considered a treatment option for PH-LD. Further large-scale studies are warranted to confirm these findings.
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Acetamidas , Hipertensión Pulmonar , Pirazinas , Humanos , Masculino , Femenino , Hipertensión Pulmonar/tratamiento farmacológico , Hipertensión Pulmonar/etiología , Hipertensión Pulmonar/fisiopatología , Anciano , Pirazinas/administración & dosificación , Pirazinas/efectos adversos , Persona de Mediana Edad , Estudios Retrospectivos , Acetamidas/administración & dosificación , Resultado del Tratamiento , Enfermedades Pulmonares/etiología , Enfermedades Pulmonares/tratamiento farmacológico , Prueba de Paso , Enfermedad Pulmonar Obstructiva Crónica/tratamiento farmacológico , Enfermedad Pulmonar Obstructiva Crónica/complicaciones , Enfermedad Pulmonar Obstructiva Crónica/fisiopatología , Enfermedades Pulmonares Intersticiales/tratamiento farmacológico , Enfermedades Pulmonares Intersticiales/etiología , Enfermedades Pulmonares Intersticiales/fisiopatología , Enfermedades Pulmonares Intersticiales/complicacionesRESUMEN
Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome is an emerging adult-onset systemic autoinflammatory disorder affecting multiple organ systems. While lung involvement is common in this syndrome, literature regarding specific patterns is sparse. In this report, we present a case description of a patient with VEXAS syndrome who presented at the emergency department on two separate occasions with acute interstitial pneumonia (AIP) and diffuse alveolar hemorrhage (DAH). A literature review with a comparison of our observed findings to the general findings of VEXAS syndrome, AIP, and DAH is provided. This report underscores the rarity of specific pulmonary manifestations associated with VEXAS syndrome, contributing valuable insight to the limited literature available on this topic.
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Hemorragia , Enfermedades Pulmonares Intersticiales , Alveolos Pulmonares , Humanos , Enfermedades Pulmonares Intersticiales/complicaciones , Enfermedades Pulmonares Intersticiales/diagnóstico por imagen , Enfermedades Pulmonares Intersticiales/diagnóstico , Alveolos Pulmonares/patología , Masculino , Enfermedades Genéticas Ligadas al Cromosoma X/genética , Enfermedades Genéticas Ligadas al Cromosoma X/complicaciones , Enfermedades Genéticas Ligadas al Cromosoma X/diagnóstico , Enfermedades Pulmonares/patología , Vacuolas/patología , Persona de Mediana Edad , Síndrome , Enzimas Activadoras de UbiquitinaAsunto(s)
Analgésicos Opioides , Disnea , Enfermedades Pulmonares Intersticiales , Humanos , Enfermedades Pulmonares Intersticiales/tratamiento farmacológico , Enfermedades Pulmonares Intersticiales/complicaciones , Analgésicos Opioides/uso terapéutico , Analgésicos Opioides/administración & dosificación , Disnea/tratamiento farmacológico , Disnea/etiología , Esquema de Medicación , Factores de Tiempo , Resultado del TratamientoRESUMEN
INTRODUCTION: Interstitial lung disease is a leading cause of mortality in patients with systemic sclerosis. Currently, there is a lack of consensus regarding screening, rescreening, diagnosis, and follow-up practices in interstitial lung disease associated with systemic sclerosis (SSc-ILD) in Colombia. METHODS: A structured survey focused on clinical practices in patients with SSc-ILD was conducted. Members of the Asociación Colombiana de Neumología y Cirugía de Tórax (Asoneumocito) and the Asociación Colombiana de Reumatología (Asoreuma) were invited to participate from March 2023 to May 2023. RESULTS: We surveyed 51 pulmonologists and 44 rheumatologists. Overall, 51.6% reported having access to multidisciplinary team discussion in ILD. Among the 95 participants, 78.9% would routinely perform a high-resolution computed tomography scan of the chest once a diagnosis of systemic sclerosis was established. This practice is more frequent among rheumatologists (84.1%) than among pulmonologists (74.5%). Approximately half of the participants would rescreen patients annually with computed tomography scan (56.8%) if baseline images were negative. Spirometry (81.1%), diffusing capacity of the lung for carbon monoxide (80.0%), and 6-min walk test (55.8%) were the most frequently performed tests upon diagnosis of systemic sclerosis. During follow-up, participants would consider repeating pulmonary function tests mostly every 6 months. CONCLUSIONS: Screening of SSc-ILD is high among pulmonologists and rheumatologists. Decision-making on diagnosis and follow-up is similar between specialties, but there are variations in their frequency and indications. Further research is needed to evaluate how to adapt recommendations for assessing SSc-ILD in different settings.
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Enfermedades Pulmonares Intersticiales , Pautas de la Práctica en Medicina , Neumólogos , Reumatólogos , Esclerodermia Sistémica , Esclerodermia Sistémica/complicaciones , Humanos , Enfermedades Pulmonares Intersticiales/etiología , Enfermedades Pulmonares Intersticiales/complicaciones , Colombia , Pautas de la Práctica en Medicina/estadística & datos numéricos , Masculino , Encuestas de Atención de la Salud , Tomografía Computarizada por Rayos X , Femenino , Persona de Mediana Edad , AdultoAsunto(s)
Analgésicos Opioides , Disnea , Enfermedades Pulmonares Intersticiales , Humanos , Enfermedades Pulmonares Intersticiales/tratamiento farmacológico , Enfermedades Pulmonares Intersticiales/complicaciones , Disnea/tratamiento farmacológico , Disnea/etiología , Analgésicos Opioides/uso terapéutico , Analgésicos Opioides/administración & dosificación , Femenino , Masculino , Persona de Mediana Edad , Esquema de Medicación , AncianoRESUMEN
Chronic breathlessness (CB) or dyspnea is prevalent in fibrotic interstitial lung diseases (F-ILD). It is the main driver of a poor health-related quality of life (HRQOL). Timely and accurate assessment and management of CB are paramount in F-ILD care. This is reflected in latest American and European guidelines that recommend early integration of symptom-targeted therapies. Despite calls for improved CB care, evidence indicates that it remains under recognized and under treated. This narrative review focuses on the current evidence for CB assessment and management in F-ILD and proposes an algorithm for patient-centered management of CB in an outpatient setting. An overview of CB assessment tools is provided along with recommendations from guidelines and experts. The limited evidence base for CB interventions in ILD is reviewed; existing dyspnea guidelines recommend a hierarchical approach to therapies starting with the implementation of nonpharmacologic interventions (NPI). Pulmonary rehabilitation is the most common NPI in F-ILD, that improves function, dyspnea, and HRQOL. Oxygen can be prescribed to treat CB associated with exertional hypoxemia early in the course of F-ILD, with evidence suggesting short-term improvements in CB and HRQOL. For patients with severe, persistent CB despite optimization of NPI and oxygen, opioids can be prescribed, initially as short-acting, low-dose oral morphine with prophylactic doses for exertion and as needed for crises. Self-management education and written action plans may help improve patient confidence and control. Development of competency in symptom management and fostering a professional and institutional culture prioritizing CB will advance patient care and should be a priority for F-ILD patients.
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Disnea , Enfermedades Pulmonares Intersticiales , Atención Dirigida al Paciente , Humanos , Disnea/terapia , Disnea/etiología , Enfermedades Pulmonares Intersticiales/complicaciones , Enfermedades Pulmonares Intersticiales/terapia , Enfermedad Crónica , Calidad de Vida , Atención AmbulatoriaRESUMEN
OBJECTIVES: Connective tissue-associated interstitial lung diseases (CTD-ILD) are believed to be caused by microvascular damage. The objective of this study was to assess the nailfold capillaroscopy (NFC) pattern in patients diagnosed with both CTD-ILD and non-CTD-ILD to identify microvascular changes and determine the relation between capillaroscopic parameters, clinical variables, and disease-related measurements. PATIENTS AND METHODS: This cross-sectional study included 95 patients with interstitial lung disease who applied to our Rheumatology and Chest Clinics between September 2021 and July 2023. The patients were divided into two groups based on their diagnosis: non-CTD-ILD (group 1) and CTD-ILD (group 2). Nailfold capillaroscopy was performed. RESULTS: Ninety-five patients, 49 (51% female, mean age 62.31 ± 11.027 years) in group 1 and 46 (69.6% female, mean age 62.09 ± 10.887 years) in group 2, were included in the study. Abnormal capillary morphologies were both detected in the CTD-ILD group and the non-CTD-ILD groups. In patients with a usual interstitial pneumonia (UIP) pattern on chest computed tomography (CT), tortuosity was higher than in patients with non-specific interstitial pneumonia (NSIP) (P = 0.041), and the proportion of tortuosity increased significantly as the duration of the disease increased (P = 0.016). CONCLUSION: Our study highlights capillaroscopic abnormalities alone may not be sufficient to differentiate CTD-ILD (other than systemic sclerosis) from non-CTD-ILD. The presence of NFC abnormalities in non-CTD-ILD may suggest that fibrotic lung disease could potentially play a role in the deterioration of the microvascular structure or abnormal angiogenesis. Our study demonstrated that a multidisciplinary approach, incorporating clinical, morphological, pathological, and serological evaluations, is necessary for interpreting ILD. Key Points ⢠Capillaroscopic abnormalities can also be seen in non-CTD-ILD. ⢠Capillaroscopy findings do not distinguish the non-Ssc etiology of ILD. ⢠Nailfold capillaroscopy may have the potential to serve as a useful tool in predicting prognosis and monitoring the disease progression in patients with idiopathic pulmonary fibrosis (IPF).
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Enfermedades Pulmonares Intersticiales , Angioscopía Microscópica , Humanos , Femenino , Enfermedades Pulmonares Intersticiales/diagnóstico por imagen , Enfermedades Pulmonares Intersticiales/complicaciones , Enfermedades Pulmonares Intersticiales/diagnóstico , Masculino , Persona de Mediana Edad , Estudios Transversales , Anciano , Pronóstico , Enfermedades del Tejido Conjuntivo/complicaciones , Enfermedades del Tejido Conjuntivo/diagnóstico por imagen , Uñas/irrigación sanguínea , Uñas/diagnóstico por imagen , Capilares/diagnóstico por imagen , Capilares/patología , Tomografía Computarizada por Rayos XRESUMEN
OBJECTIVES: This study aimed to investigate the clinical relevance of antineutrophil cytoplasmic antibody (ANCA) in patients with rheumatoid arthritis-associated interstitial lung disease (RA-ILD). METHODS: Detailed clinical records of rheumatoid arthritis (RA) patients who underwent ANCA screening tests were collected. ANCA measurements were determined by indirect immunofluorescence assay (IIF) and enzyme-linked immunosorbent assay (ELISA). Clinical characteristics were compared between ANCA-positive and ANCA-negative groups, and multivariable logistic models were used to evaluate the independent association of ANCA with ILD in RA patients. RESULTS: The prevalence of ANCA by IIF was significantly higher in RA-ILD patients compared to those with RA without ILD (31.7 % vs. 19.5 %, p < 0.001). RA-ILD patients positive for ANCA exhibited elevated levels of inflammatory markers and greater disease activity, and showed more severe impairment of lung function compared to ANCA-negative RA-ILD patients. Multivariable logistic regression analysis revealed an independent association of ANCA, especially pANCA, with RA-ILD. ANCA specificities for BPI, elastase, and cathepsin-G were found in 15.6 % of RA-ILD patients; the specificities for most others remain unknown. CONCLUSIONS: The findings suggest a potential role for ANCA/pANCA in stratifying the risk of RA and provide supplementary information to the existing clinically available assays. This additional information may be valuable in identifying RA patients who require further investigations for RA-ILD, such as high-resolution computed tomography (HRCT). These results emphasize the potential clinical relevance of ANCA in the context of RA-ILD.
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Anticuerpos Anticitoplasma de Neutrófilos , Artritis Reumatoide , Enfermedades Pulmonares Intersticiales , Humanos , Enfermedades Pulmonares Intersticiales/inmunología , Enfermedades Pulmonares Intersticiales/complicaciones , Artritis Reumatoide/complicaciones , Artritis Reumatoide/inmunología , Artritis Reumatoide/sangre , Masculino , Femenino , Anticuerpos Anticitoplasma de Neutrófilos/sangre , Anticuerpos Anticitoplasma de Neutrófilos/inmunología , Persona de Mediana Edad , Factores de Riesgo , AncianoRESUMEN
BACKGROUND: Pulmonary hypertension due to interstitial lung disease (PH-ILD) is associated with high rates of respiratory failure and death. Healthcare resource utilization (HCRU) and cost data are needed to characterize PH-ILD disease burden. METHODS: A retrospective cohort analysis of the Truven Health MarketScan® Commercial Claims and Encounters Database and Medicare Supplemental Database between June 2015 to June 2019 was conducted. Patients with ILD were identified and indexed based on their first claim with a PH diagnosis. Patients were required to be 18 years of age on the index date and continuously enrolled for 12-months pre- and post-index. Patients were excluded for having a PH diagnosis prior to ILD diagnosis or the presence of other non-ILD, PH-associated conditions. Treatment patterns, HCRU, and healthcare costs were compared between the 12 months pre- versus 12 months post-index date. RESULTS: In total, 122 patients with PH-ILD were included (mean [SD] age, 63.7 [16.6] years; female, 64.8%). The same medication classes were most frequently used both pre- and post-index (corticosteroids: pre-index 43.4%, post-index 53.5%; calcium channel blockers: 25.4%, 36.9%; oxygen: 12.3%, 25.4%). All-cause hospitalizations increased 2-fold, with 29.5% of patients hospitalized pre-index vs. 59.0% post-index (P < 0.0001). Intensive care unit (ICU) utilization increased from 6.6 to 17.2% (P = 0.0433). Mean inpatient visits increased from 0.5 (SD, 0.9) to 1.1 (1.3) (P < 0.0001); length of stay (days) increased from 5.4 (5.9) to 7.5 (11.6) (P < 0.0001); bed days from 2.5 (6.6) to 8.0 (16.3) (P < 0.0001); ICU days from 3.8 (2.3) to 7.0 (13.2) (P = 0.0362); and outpatient visits from 24.5 (16.8) to 32.9 (21.8) (P < 0.0001). Mean (SD) total all-cause healthcare costs increased from $43,201 ($98,604) pre-index to $108,387 ($190,673) post-index (P < 0.0001); this was largely driven by hospitalizations (which increased from a mean [SD] of $13,133 [$28,752] to $63,218 [$75,639] [P < 0.0001]) and outpatient costs ($16,150 [$75,639] to $25,604 [$93,964] [P < 0.0001]). CONCLUSION: PH-ILD contributes to a high HCRU and cost burden. Timely identification, management, and treatment are needed to mitigate the clinical and economic consequences of PH-ILD development and progression.
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Costo de Enfermedad , Costos de la Atención en Salud , Hipertensión Pulmonar , Enfermedades Pulmonares Intersticiales , Humanos , Enfermedades Pulmonares Intersticiales/economía , Enfermedades Pulmonares Intersticiales/complicaciones , Femenino , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Anciano , Hipertensión Pulmonar/economía , Hipertensión Pulmonar/terapia , Hipertensión Pulmonar/epidemiología , Costos de la Atención en Salud/estadística & datos numéricos , Estados Unidos , Adulto , Hospitalización/economía , Hospitalización/estadística & datos numéricos , Aceptación de la Atención de Salud/estadística & datos numéricos , Anciano de 80 o más Años , Bases de Datos FactualesRESUMEN
INTRODUCTION: Cough is common in interstitial lung disease (ILD) and is associated with disease progression, yet its mechanisms are understudied. We investigated cough hypersensitivity features and impact in ILD. METHODS: Participants with ILD and cough (n = 195) completed a multiple choice and free text questionnaire on cough sensations/triggers and impacts. RESULTS: The majority of participants were male (54%), aged > 65 (64%), with idiopathic pulmonary fibrosis (IPF, 75%). Common cough triggers were body position (74%), physical activity (72%), and talking (62%). Common laryngeal sensations were globus (43%), and itch/tickle (42%). Cough impacted everyday life in 55%, and all activities in 31%, causing exhaustion (59%), social embarrassment (70%), urinary incontinence (46% females), and syncope/pre-syncope (12%). The total number of cough-provoking sensations/triggers correlated with impacts; ρ = 0.73, p < 0.001. CONCLUSION: Cough hypersensitivity symptoms are prevalent in ILD and detrimentally affect quality of life. Further studies investigating mechanisms of cough hypersensitivity and targeted pharmacotherapy are warranted.
Asunto(s)
Tos , Enfermedades Pulmonares Intersticiales , Calidad de Vida , Humanos , Tos/psicología , Tos/fisiopatología , Masculino , Femenino , Anciano , Enfermedades Pulmonares Intersticiales/psicología , Enfermedades Pulmonares Intersticiales/complicaciones , Enfermedades Pulmonares Intersticiales/fisiopatología , Persona de Mediana Edad , Encuestas y Cuestionarios , Percepción , Fibrosis Pulmonar Idiopática/complicaciones , Fibrosis Pulmonar Idiopática/fisiopatología , Fibrosis Pulmonar Idiopática/psicología , Síncope/fisiopatología , Síncope/etiología , Actividades CotidianasRESUMEN
RATIONALE: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that damages multiple organs and systems, including the lungs, kidneys, and heart. The respiratory system is commonly affected by SLE, leading to problems such as pleurisy, pleural effusion, and interstitial lung disease (ILD). In addition, SLE can involve the heart, with pericarditis being the most common manifestation. Notably, pericardial effusion frequently accompanies pericarditis involved by SLE, and aspects such as thickened pericardium (TP) can be challenging to detect early on. There are limited reports on TP and even fewer reports on the treatment of ILD with TP. This study investigates the clinical treatment of SLE complicating ILD and TP and reports on a successful case treated with tofacitinib, offering new strategies for managing such patients. PATIENT CONCERNS: A 35-year-old female patient presented to the hospital with polyarticular swelling and pain that had been ongoing for over 4 years, as well as recurrent chest pain for 2 years that worsened over the course of 1 day. DIAGNOSES: The patient was diagnosed with SLE complicating ILD and TP, with hematologic involvement. INTERVENTIONS: Treatment involved the administration of tofacitinib in combination with low-dose methylprednisolone (MP) and mycophenolate mofetil (MMF). OUTCOMES: The patient experienced recurrent chest pain and difficulty in reducing glucocorticoids (GCs), but the patient conditions were improved upon the addition of tofacitinib. The patient has been followed up for 16 months, and the patient MP dosage has been reduced to 6 mg once daily. The patient condition remains stable without recurrence, and the patient quality of life has improved. LESSONS: In cases of SLE complicating ILD and TP, when tapering GCs is difficult, treatment with tofacitinib can be effective in achieving remission and maintaining stability.
Asunto(s)
Enfermedades Pulmonares Intersticiales , Lupus Eritematoso Sistémico , Piperidinas , Pirimidinas , Humanos , Pirimidinas/uso terapéutico , Piperidinas/uso terapéutico , Piperidinas/administración & dosificación , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/tratamiento farmacológico , Femenino , Enfermedades Pulmonares Intersticiales/tratamiento farmacológico , Enfermedades Pulmonares Intersticiales/complicaciones , Enfermedades Pulmonares Intersticiales/etiología , Adulto , Pericardio/patología , Inhibidores de Proteínas Quinasas/uso terapéutico , Inhibidores de Proteínas Quinasas/administración & dosificación , Ácido Micofenólico/uso terapéutico , Metilprednisolona/uso terapéutico , Metilprednisolona/administración & dosificaciónRESUMEN
PURPOSE OF REVIEW: To review the current understanding of the impact, mechanisms and treatments for cough in patients with interstitial lung disease (ILD). Evidence suggests that cough is a prevalent symptom in patients with ILD and has a significant impact on patients. RECENT FINDINGS: There is increasing interest in the role of cough hypersensitivity as seen in chronic refractory cough in patients with ILD, and encouraging recent results suggest that ILD-associated cough responds to opiate therapy. SUMMARY: Understanding the aetiology of cough in patients with ILD is crucial to continue to develop therapies which might be effective in reducing cough and increasing quality of life.