Lung ailments in rheumatic diseases present unique challenges for diagnosis and management and are a source of significant morbidity and mortality for patients. Unlike the idiopathic interstitial ...pneumonias, patients with rheumatic diseases experience lung disease in the context of a systemic disease that may make it more difficult to recognize and that may present greater risks with treatment. Despite recent advances in our awareness of these diseases, there is still a significant lack of understanding of natural history to elucidate which patients will have disease that is progressive and thus warrants treatment. What we do know is that a subset of patients with rheumatic disease experience parenchymal lung disease that can prognostically resemble idiopathic pulmonary fibrosis, such as in rheumatoid arthritis, and that others can have aggressive inflammatory lung disease in the context of autoimmune myositis, systemic sclerosis, or an undifferentiated autoimmune process. As we enter into a paradigm shift where we view lung health as a cornerstone of our care of patients with rheumatic diseases, we hopefully will improve our ability to identify those patients at highest risk for pulmonary disease and progression, and offer emerging treatments which will result in better outcomes and a better quality of life.
Objective
To evaluate rheumatoid arthritis (RA) disease activity and risk of RA‐associated interstitial lung disease (RA‐ILD).
Methods
We investigated disease activity and risk of RA‐ILD using the ...Brigham RA Sequential Study (BRASS, 2003–2016). All patients were diagnosed as having RA according to accepted criteria. Disease Activity Scores in 28 joints (DAS28) and covariate data were measured prospectively at annual study visits. Diagnosis of RA‐ILD was determined by review of images from clinically indicated chest computed tomography scans. We analyzed patients without RA‐ILD at baseline. We used Cox regression to estimate hazard ratios (HRs) and 95% confidence intervals (CIs) for RA‐ILD, using annually updated DAS28 data, with adjustment for known RA‐ILD risk factors (age, sex, smoking status, RA duration, and serologic status). We performed alternative analyses that did not censor at the time of missing DAS28 data and included adjustment for use of methotrexate, use of glucocorticoids, presence of bone erosions, and presence of rheumatoid nodules.
Results
Among 1,419 participants, the mean ± SD age was 55.8 ± 14.2 years, and 68.6% were seropositive for either cyclic citrullinated peptide or rheumatoid factor. We identified 85 incident cases of RA‐ILD during a mean ± SD follow‐up duration of 8.9 ± 4.2 years per patient. The moderate/high disease activity group had a multivariable HR of 2.22 (95% CI 1.28–3.82) for RA‐ILD compared to the remission/low disease activity group. Risk of RA‐ILD increased across disease activity categories: multivariable HR 1.00 (reference) for remission, 1.41 (95% CI 0.61–3.28) for low disease activity, 2.08 (95% CI 1.06–4.05) for moderate disease activity, and 3.48 (95% CI 1.64–7.38) for high disease activity (P for trend = 0.001). For each unit increase in the DAS28, the risk of RA‐ILD increased by 35% (95% CI 14–60%). Results were similar in analyses that included follow‐up for missing DAS28 data and with adjustment for use of methotrexate, use of glucocorticoids, presence of bone erosions, or presence of rheumatoid nodules.
Conclusion
Active articular RA was associated with an increased risk of developing RA‐ILD. These results suggest that decreasing systemic inflammation may alter the natural history of RA‐ILD development.
Rheumatoid arthritis (RA) is commonly associated with pulmonary disease that can affect any anatomic compartment of the thorax. The most common intrathoracic manifestations of RA include interstitial ...lung disease, airway disease, pleural disease, rheumatoid nodules, and drug-induced toxicity. Patients with RA with thoracic involvement often present with nonspecific respiratory symptoms, although many are asymptomatic. Therefore, clinicians should routinely consider pulmonary disease when evaluating any patient with RA, particularly one with known risk factors. The optimal screening, diagnostic, and treatment strategies for RA-associated pulmonary disease remain uncertain and are the focus of ongoing investigation.
Interstitial lung disease (ILD) in the connective tissue diseases (CTD) is amongst the most challenging aspect of care of patients with rheumatic diseases and is the source of significant morbidity ...and mortality. While there has been progress in our understanding of the natural history of these complications, we still suffer from a limited reservoir of data to confidently determine which patients are at highest risk for disease and those who are at highest risk for disease progression. Treatment options until recently have been limited to anti-inflammatory therapies but with the emerging availability of anti-fibrotic therapies, a shift in strategy is emerging to target therapies based on the specific radiographic, histopathologic features and biomarker profiles that are unique to patients with rheumatic diseases and ILD.
Idiopathic pulmonary fibrosis (IPF) is a progressive, fatal interstitial lung disease (ILD) characterized by abnormal extracellular matrix (ECM) remodeling. We hypothesized that ECM remodeling might ...result in a plasma profile of proteins specific for IPF that could distinguish patients with IPF from other idiopathic ILDs.
To identify biomarkers that might assist in distinguishing IPF from non-IPF ILD.
We developed a panel of 35 ECM, ECM-related, and lung-specific analytes measured in plasma from 86 patients with IPF (derivation cohort) and in 63 patients with IPF (validation cohort). Comparison groups included patients with rheumatoid arthritis-associated ILD (RA-ILD; n = 33), patients with alternative idiopathic ILDs (a-ILD; n = 41), and healthy control subjects (n = 127). Univariable and multivariable logistic regression models identified biomarkers that differentiated patients with IPF from those with a-ILD. Both continuous and diagnostic threshold versions of biomarkers were considered; thresholds were chosen to maximize summed diagnostic sensitivity and specificity in univariate receiver-operating characteristic curve analysis. A diagnostic score was created from the most promising analytes.
Plasma surfactant protein (SP)-D > 31 ng/ml, matrix metalloproteinase (MMP)-7 > 1.75 ng/ml, and osteopontin > 6 ng/ml each significantly distinguished patients with IPF from patients with a-ILD, both individually and in a combined index. The odds ratio for IPF when at least one analyte in the index exceeded the threshold was 4.4 (95% confidence interval, 2.0-9.7; P = 0.0003). When at least two analytes were elevated, the odds ratio for IPF increased to 5.0 (95% confidence interval, 2.2-11.5; P = 0.0002).
A biomarker index of SP-D, MMP-7, and osteopontin enhanced diagnostic accuracy in patients with IPF compared with those with non-IPF ILD. Our data suggest that this biomarker index may improve diagnostic confidence in IPF.
To evaluate methods of identifying patients with systemic sclerosis (SSc) using International Classification of Diseases, Tenth Revision (ICD-10) codes (M34*), electronic health record (EHR) ...databases and organ involvement keywords, that result in a validated cohort comprised of true cases with high disease burden.
We retrospectively studied patients in a healthcare system likely to have SSc. Using structured EHR data from January 2016 to June 2021, we identified 955 adult patients with M34* documented 2 or more times during the study period. A random subset of 100 patients was selected to validate the ICD-10 code for its positive predictive value (PPV). The dataset was then divided into a training and validation sets for unstructured text processing (UTP) search algorithms, two of which were created using keywords for Raynaud's syndrome, and esophageal involvement/symptoms.
Among 955 patients, the average age was 60. Most patients (84%) were female; 75% of patients were White, and 5.2% were Black. There were approximately 175 patients per year with the code newly documented, overall 24% had an ICD-10 code for esophageal disease, and 13.4% for pulmonary hypertension. The baseline PPV was 78%, which improved to 84% with UTP, identifying 788 patients likely to have SSc. After the ICD-10 code was placed, 63% of patients had a rheumatology office visit. Patients identified by the UTP search algorithm were more likely to have increased healthcare utilization (ICD-10 codes 4 or more times 84.1% vs 61.7%, p < .001), organ involvement (pulmonary hypertension 12.7% vs 6% p = .011) and medication use (mycophenolate use 28.7% vs 11.4%, p < .001) than those identified by the ICD codes alone.
EHRs can be used to identify patients with SSc. Using unstructured text processing keyword searches for SSc clinical manifestations improved the PPV of ICD-10 codes alone and identified a group of patients most likely to have SSc and increased healthcare needs.
Celotno besedilo
Dostopno za:
DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, SIK, UILJ, UKNU, UL, UM, UPUK
Sarcoidosis is a systemic disease characterized by the development of epithelioid granulomas in various organs. Although the lungs are involved in most patients with sarcoidosis, virtually any organ ...can be affected. Recognition of extrapulmonary sarcoidosis requires awareness of the organs most commonly affected, such as the skin and the eyes, and vigilance for the most dangerous manifestations, such as cardiac and neurologic involvement. In this article, the common extrapulmonary manifestations of sarcoidosis are reviewed and organ-specific therapeutic considerations are discussed.
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