Sarcoidosis, Vasculitis, and Diffuse Lung Diseases最新文献

Background and aim: Patient related outcomes are important in sarcoidosis but the medium-term repeatability of the key patient reported outcome measure is not known. We aimed to test the repeatability of the Fatigue Assessment Scale (FAS), Short Form 6-Dimension (SF-6D), and King's Sarcoidosis Questionnaire (KSQ) in free living people with sarcoidosis associated fatigue.

Methods: Twelve people with sarcoidosis associated fatigue completed the FAS, short form 36 questionnaire (SF-36) and the KSQ at baseline and 12 weeks. The SF-6D utility was calculated from the SF-36. The difference between baseline and 12 week assessments was measured.

Results: The interclass correlation (95% confidence interval) showed good agreement between the baseline and 3 months measurements: FAS 0.91 (0.74, 0.71), SF-36 0.98 (0.94, 1), KSQ 0.98 (0.93, 0.99), SF-6D utility 0.98 (0.93, 0.99). The baseline (standard deviation) FAS was 27.83 (5.86) and at 12 weeks was 27.25 (7.55) representing 0.58 difference (95% CI for difference (-1.89, 3.06)), SF-6D utility was 0.69 (0.16) at baseline and 0.68 (0.17) after 3 months representing at 0.00 (-0.03, 0.03) difference and corresponding values for KSQ were 59.12 (18.68) and 56.91 (27.26) with a difference of -1.87 (5.49,1.76).

Conclusions: There was good repeatability of FAS, SF-36, SF-6D and KSQ in free living people with sarcoidosis associated fatigue. Fatigue, general and disease specific health related quality of life showed no significant change over a 12 week period. Studies identifying changes in these outcomes can confidently report a true change and not measurement error or regression to the mean.

.

Background and aim:  Pulmonary artery aneurysms (PAAs) are the most well-defined type of pulmonary vascular complication in Behçet's disease (BD).The aim of this study is to analyze which CT pulmonary angiography (CTPA) signs are associated with serious morbidity and mortality.

Methods: The study included 42 BD patients with pulmonary vascular complications. All patients' medical records were reviewed retrospectively in terms of demographics, disease characteristics, laboratory investigations, pulmonary manifestations, arterial and/or venous thrombosis and CTPA vascular and parenchymal findings.

Results: Deep venous thrombosis was observed in 31(73.8%) patients, arterial thrombosis in 13(31%), peripheral arterial aneurysms in 12(286%), haemoptysis in 38 (90.5%), and fatal haemoptysis in 8(19 %) patients. CTPA revealed: in situ thrombosis in 14(33.3%) patients, true stable PAAs in 13(31), true unstable PAAs in 11(26.2%), stable pulmonary artery pseudoaneurysms (PAPs) in 7(16.7%), unstable PAPs in 17(40.5%), the latter were associated with perianeurysmal leaking in 26(61.9%) and bronchial indentation in 19(45.2%).In regression analysis, fatal outcomes were associated with age in years (p=0.035), arterial thrombosis (p=0.025), peripheral arterial aneurysms (p=0.010), intracardiac thrombosis (p=0.026) and positively associated with haemoptysis severity (p<0.001).

Conclusion: Peripheral arterial thrombosis and/or aneurysms, intracardiac thrombosis and haemoptysis severity are predictor of fatal outcomes in BD pulmonary vasculitis. PAPs with perianeurysmal alveolar haemorrhage and/or bronchial indentation are serious CTPA signs that require prompt identification and aggressive treatment. PAPs are a more serious aneurysmal pattern than true PAAs because they are a contained rupture of a PA branch in the context of pulmonary vasculitis.

Background and aim: Cardiac sarcoidosis (CS) is the second most common cause of death in patients with sarcoidosis and data pertaining to its diagnosis and management is limited. We sought to describe diagnostic modalities and management of patients with CS in the United States, based on a national registry questionnaire.

Methods: We conducted a retrospective study based on a national registry investigating 3,835 respondents to the Foundation for Sarcoidosis Research Questionnaire. The registry includes patient surveys completed between June 2014 and August 2019. Summary and univariate analyses were performed.

Results: A total of 394 patients (10.3%) with CS were identified; 57% (n=223) were women and 81% (n=317) were white. The mean (±SD) age at diagnosis was 45 years (±13). CS was the initial presentation of sarcoidosis in 30%. Multiorgan involvement (≥3 organs) was present in 68%. Two-thirds of patients were admitted at least once to the hospital. Cardiac magnetic resonance imaging (74.4%) was the most common diagnostic modality used followed by positron emission tomography (PET) scan (59.3%) and cardiac biopsy (n=52, 13%).  Most patients received corticosteroids (86%) and steroid-sparing medications (61%) including methotrexate (26%) and tumor necrosis factor (TNF) inhibitors (19%). A combined cardioverter defibrillator and pacemaker (39%) was the most common cardiac device implanted.

Conclusions: The prevalence of CS in this cohort was higher than previously described. CS was a common initial presentation of sarcoidosis. The diagnosis was most likely made using cMRI. Steroids, methotrexate and infliximab are the most common medications used. Conduction abnormalities and arrhythmias often occurred.

We have described a clinical case with atypical onset of sarcoidosis. A young patient presented to the emergency room with acute heart failure and severe cardiac dysfunction simulating dilated cardiomyopathy or severe myocarditis. Hypoxic respiratory failure refractory to oxygen therapy was treated with steroids. The diagnosis was made by a multidisciplinary team who decided to perform EndoBronchial UltraSound-guided TransBronchial Needle Aspiration (EBUS TBNA) in addition to a cardiac MRI. The result was to obtain, in this case, a prompt therapeutic response in order to preserve the patient cardiac function.

Background: We aimed to evaluate the pulmonary involvement status, its related factors, and pulmonary function test (PFT) results in the first month follow-up in patients who were discharged for severe Covid-19 pneumonia, and to assess the efficacy of corticosteroid treatment on these parameters in severe pulmonary involvement patients.

Methods: We retrospectively analyzed all consecutive patients who applied to our COVID-19 follow-up clinic at the end of the first month of hospital discharge. Functional and radiological differences were compared after 3 months of corticosteroid treatment in severe pulmonary involvement group. Results We analyzed 391 patients with "pulmonary parenchymal involvement" (PPIG) and 162 patients with "normal lung radiology" (NLRG). 122 patients in the PPIG (corticosteroid-required interstitial lung disease group (CRILD)) had severe pulmonary involvement with frequent symptoms and required corticosteroid prescription. Pulmonary involvement was more common in males and elder patients (P<0.001, for both). Being smoker and elderly were associated with a higher risk-ratio in predicting to be in PPIG (OR:2.250 and OR:1.057, respectively). Smokers, male and elderly patients, and HFNO2 support during hospitalization were risk factors for being a patient with CRILD (OR:2.737, OR:4.937, OR:4.756, and OR:2.872, respectively). After a three-months of methylprednisolone medication, a good response was achieved on radiological findings and PFT results in CRILD.

Conclusions: In conclusion, after severe COVID-19 pneumonia, persistent clinical symptoms and pulmonary parenchymal involvement would be inevitable in elder and smoker patients. Moreover, corticosteroid treatment in patients with severe parenchymal involvement was found to be effective in the improvement of radiological and functional parameters.

Background: Little is known about pulmonary hypertension (PH) in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).

Objectives: The aims of this retrospective study, in which echocardiography was used for detection of PH, were to identify the potential causes of PH in AAV and to analyze the risk factors for mortality.

Methods: We performed a retrospective descriptive review of 97 patients who had AAV with PH at our institution from January 1, 1997, through December 31, 2015. These patients with PH were compared with 558 patients who had AAV without PH. Demographic and clinical data were abstracted from electronic health records.

Results: Among the patients who had PH, 61% were men; mean (SD) age was 70.5 (14.1) years at the time of PH diagnosis. The majority of patients with PH (73.2%) had more than 1 potential cause of PH, with left heart disease and chronic lung disease being the most common causes. Older age, male sex, smoking history, and kidney involvement were associated with the presence of PH. PH was associated with an increased risk of death (hazard ratio, 3.15; 95% CI, 2.37-4.18). On multivariate analysis, PH, age, smoking status, and kidney involvement were independent risk factors for death. Median survival after the diagnosis of PH was 25.9 months (95% CI, 12.2-49.9).

Conclusions: PH in AAV is often multifactorial, is commonly associated with left heart disease, and is associated with a poor prognosis.

Sarcoidosis is a multisystemic granulomatous disease of unknown origin. It has been argued that the skin is one of the entry doors of the possible antigen that causes sarcoidosis and after entering the skin, the causal agent may progress to the underlying bone. We report four cases with development of sarcoidosis in old scars located on the forehead, and contiguous bone involvement of the frontal bone. In most cases scar sarcoidosis was the first manifestation of the disease, and in most cases it was asymptomatic. Two patients never required treatment, and in all cases the frontal problem improved or remained stable spontaneously or under sarcoidosis treatment. Scar sarcoidosis in the frontal area may have contiguous bone damage. This bone involvement does not seem to be associated with neurological extension.

Background: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare autoimmune disease usually involving small vessels and progressing with necrotizing inflammation. Treatment requires long-term use of immunosuppressive agents to inhibit disease activity. Serious infections (SIs) are a common complication in AAV.

Objective: The aim of this study was to identify the risk factors for serious infections which required hospitalization in patients with AAV.

Methods: In this retrospective cohort study., we included 84 patients admitted to the Ankara University Faculty of Medicine in the last 10 years with a diagnosis of AAV.

Results: In 42 (50%) of 84 patients followed up with the diagnosis of AAV, an infection requiring hospitalization was identified. The patients' total corticosteroid dose, use of pulse steroids, induction regimen, levels of C-reactive protein (CRP) and the presence of pulmonary and renopulmonary involvement were found to be associated with the frequency of infection (p=0.015, p=0.016, p=0.010, p=0.03, p= 0.026 and p=0.029, respectively). In multivariable analysis, it was found that renopulmonary involvement (p=0.002, HR=4.95, 95% CI= 1.804-13.605), age of over 65 (p=0.049, HR=3.37, 95% CI=1.004-11.369) and high CRP levels (p=0.043, HR=1.006, 95% CI=1.000-1.011) constituted independent predictors of serious infection risk.

Conclusion: The frequency of infection is known to be increased in ANCA-associated vasculitis. Our study showed that renopulmonary involvement, age and elevated CRP levels on admission are independent risk factors of infection.

Background: Krebs von den Lungen-6 (KL-6) is a high molecular weight (MW) glycoprotein mainly secreted by type II pneumocytes because of lung damage or during regeneration. Neurosarcoidosis (NS), where sarcoid granulomas involve the nervous system, occurs in 5-20% of patients with sarcoidosis. No data is currently available on KL-6 in serum or CSF of NS patients. The present study compared KL-6 concentrations in serum and CSF of NS patients versus others with neurodegenerative (ND) or chronic inflammatory demyelinating (DM) diseases.

Materials and methods: Nine NS patients (mean age 46.2 years, range 16-61 years, M/F 5/4), nine patients with a chronic neurodegenerative disease (mean age 53.1 years, range 37-65 years, M/F 5/4) and nine patients with a chronic demyelinating disease (mean age 46.3 years, range 18-65 years, M/F 5/4) were retrospectively enrolled.

Results: Measurable CSF concentrations of KL-6 were detected in 7/9 NS patients but in no ND or DM patients. No significant differences in CSF concentrations of ACE were observed between the three groups (p=0.0819). In NS patients, CSF concentrations of KL-6 were directly correlated with CSF albumin index (r=0.98; p<0.0001), albumin (r=0.979, p=0.0001), IgG (r=0.928, p=0.0009) and total protein concentrations (r=0.945, p=0.0004).

Discussion: KL-6 is a high MW protein, under physiological conditions it is unlikely to cross the blood-brain barrier. We found KL-6 in CSF from NS and not from ND and DM patients. The finding sustains the specificity of changes in KL-6 in this granulomatous disease, suggesting it as a candidate biomarker for recognition of NS.