Is There a Link Between Nailfold Videocapillaroscopy and Pulmonary Function Tests in Systemic Sclerosis Patients?: A 24-Month Follow-up Monocentric Study.

BACKGROUND/OBJECTIVE: The aim of this study was to explore the associations between nailfold videocapillaroscopy (NVC) and pulmonary function tests (PFTs) in systemic sclerosis (SSc) patients. METHODS: This was a longitudinal study with follow-up of unselected Brazilian SSc patients. Baseline clinical examination, serological workup, high-resolution chest tomography, and NVC were performed. Pulmonary function test was performed at baseline and after 24 months. Pulmonary function test worsening over time was defined as either a ΔFVC decline ≥10% or a ΔFVC decline ≥5% and <9%, combined with a ΔDLCO decline ≥15%, at 24 months. The NVC parameters were quantitatively (capillary density number, dimension, morphology, and hemorrhages) and qualitatively (NVC pattern) evaluated according to the consented standardized definitions of the EULAR Study Group on Microcirculation in Rheumatic Diseases. RESULTS: Seventy-nine patients were included. Fifty-nine were rated to have a scleroderma pattern (6 "early"/16 "active"/37 "late"). The mean FVC and DLCO were 76.8% and 67.2% at baseline and 73.8% and 64.3% at 24 months, respectively. After multivariate analysis, we found that a reduced baseline FVC was associated with reduced capillary density (odds ratio [OR], 11; 95% confidence interval [CI], 1.5-90.7; p = 0.03) and a reduced baseline DLCO associated with the late scleroderma pattern (OR, 6.75; 95% CI, 1.09-42; p = 0.03). No association between worsening of PFT over time and NVC was found. CONCLUSIONS: The association between NVC and PFTs might corroborate the link between microangiopathy and interstitial lung disease in patients with SSc. This finding might strengthen the idea of incorporating NVC as a tool to predict progressive interstitial lung disease in these patients in the future.

Pulmonary hypertension in systemic sclerosis: diagnosis by systematic screening and prognosis after three years follow-up.

BACKGROUND: Systemic sclerosis (SSc) is a rare disease, and the presence of pulmonary hypertension can be a determining factor in prognosis. The aim of this study was to evaluate the diagnosis, profile, and prognosis of systemic sclerosis pulmonary hypertension (SSc-PH) diagnosed by systematic screening in a Brazilian population. METHODS: A cohort of SSc patients underwent systematic screening for SSc-PH. Patients were referred for right heart catheterization (RHC) according to transthoracic echocardiogram or a combination of diagnostic tools. The clinical, immunological, and hemodynamic features and prognosis after 3 years were evaluated. RESULTS: Twenty patients underwent RHC. SSc pulmonary arterial hypertension (SSc-PAH) was the most common group of SSc-PH. These patients had long disease duration, high urate levels and highly elevated mean pulmonary arterial pressure (mPAP) and peripheral vascular resistance (PVR) on hemodynamics. Patients with mPAP > 20- < 25 mmHg had hemodynamic features of intermediate disease. Patients with SSc-PH associated to interstitial lung disease (SSc-ILD-PH) had signs of vasculopathy on hemodynamics. In patients with no-SSc-PH, the survival at 1, 2, and 3 years was 96%, 92% and 92%, respectively and in patients with SSc-PH it was 86.7%, 60% and 53.3%, respectively. CONCLUSIONS: Patients identified with SSc-PAH and SSc-ILD-PH in our screening had severe clinical and hemodynamic features. Mortality remains high in SSc-PH but was more related to Bo-PAH and SSc-ILD-PH, while in SSc-PAH, the prognosis was better. TRIAL REGISTRATION: Current Controlled Trials ISRCTN 72968188, July 8th, 2021. Retrospectively registered.

Defining prognosis in sarcoidosis.

Sarcoidosis is a multi-systemic granulomatous disease. Affected individuals can show spontaneous healing, develop remission with drug treatment within 2 years, or become chronically ill. Our main goal was to identify features that are related to prognosis.The study consisted of 101 patients, recruited at a single center, who were already diagnosed with sarcoidosis at the start of the study or were diagnosed within 48 months. Ninety individuals were followed-up for at least 24 months and were classified according to clinical outcome status (COS 1 to 9). Those with COS 1-4 and COS 5-9 were classified as having favorable and unfavorable outcomes, respectively. Unconditional logistic regression analyses were conducted to define which variables were associated with sarcoidosis outcomes. Subsequently, we established a scoring system to help predict the likelihood of a favorable or unfavorable outcome.Of our patients, 48% developed a chronic form of the disease (COS 5-9). Three clinical features were predictive of prognosis in sarcoidosis. We built a score-based model where the absence of rheumatological markers (1 point), normal pulmonary functions (2 points), and the presence of early respiratory symptoms manifestations (2 points) were associated with a favorable prognosis. We predicted that a patient with a score of 5 had an 86% (95% confidence interval [CI] 74%-98%) probability of having a favorable prognosis, while those with scores of 4, 3, 2, 1, and 0 had probabilities of 72% (95% CI 59-85%), 52% (95% CI 40-63%), 31% (95% CI 17-44%), 15% (95% CI 2-28%), and 7% (95% CI 0-16%) of having a favorable prognosis, respectively. Thus, our easy-to-compute algorithm can help to predict prognosis of sarcoidosis patients, facilitating their management.

Automatic Quantification of Interstitial Lung Disease From Chest Computed Tomography in Systemic Sclerosis.

Background: Interstitial lung disease (ILD) is a common complication in patients with systemic sclerosis (SSc), and its diagnosis contributes to early treatment decisions. Purposes: To quantify ILD associated with SSc (SSc-ILD) from chest CT images using an automatic quantification method based on the computation of the weight of interstitial lung opacities. Methods: Ninety-four patients with SSc underwent CT, forced vital capacity (FVC), and carbon monoxide diffusion capacity (DLCO) tests. Seventy-three healthy individuals without radiological evidence of lung disease served as controls. After lung and airway segmentation, the ratio between the weight of interstitial opacities [densities between -500 and +50 Hounsfield units (HU)] and the total lung weight (densities between -1,000 and +50 HU) was used as an ILD indicator (ILD[%] = 100 × [LW(-500 to +50HU)/LW(-1, 000 to +50HU)]). The cutoff of normality between controls and SSc was determined with a receiver operator characteristic curve. The severity of pulmonary involvement in SSc patients was also assessed by calculating Z scores of ILD relative to the average interstitial opacities in controls. Accordingly, SSc-ILD was classified as SSc Limited-ILD (Z score < 3) and SSc Extensive-ILD (Z score ≥ 3 or FVC < 70%). Results: Seventy-eight (83%) SSc patients were classified as presenting SSc-ILD (optimal ILD threshold of 23.4%, 0.83 sensitivity, 0.92 specificity, and 0.94 area under the receiver operator characteristic curve, 95% CI from 0.89 to 0.96, 0.93 positive predictive value, and 0.81 negative predictive value, p < 0.001) and exhibited radiological attenuations compatible with interstitial pneumonia dispersed in the lung parenchyma. Thirty-six (38%) patients were classified as SSc Extensive-ILD (ILD threshold ≥ 29.6% equivalent to a Z score ≥ 3) and 42 (45%) as SSc Limited-ILD. Eighteen (50%) patients with SSc Extensive-ILD presented FVC < 70%, being only five patients classified exclusively based on FVC. SSc Extensive-ILD also presented lower DLCO (57.9 ± 17.9% vs. 73.7 ± 19.8%; p < 0.001) and total lung volume (2,916 ± 674 vs. 4,286 ± 1,136, p < 0.001) compared with SSc Limited-ILD. Conclusion: The proposed method seems to provide an alternative to identify and quantify the extension of ILD in patients with SSc, mitigating the subjectivity of semiquantitative analyzes based on visual scores.

Changes in lung ultrasound of symptomatic healthcare professionals with COVID-19 pneumonia and their association with clinical findings.

PURPOSE: To evaluate ultrasound signs of coronavirus disease-19 (COVID-19) pneumonia in symptomatic healthcare professionals and to correlate those changes with clinical findings. METHODS: All patients underwent real-time polymerase chain reaction (RT-PCR), lung ultrasound (LUS) and clinical evaluation on the same day. In each of the 12 areas evaluated in the LUS, the LUS signs were scored to generate the aeration score. RESULTS: A total of 409 participants had positive PCR, with a median age of 41 (35-51) years. All participants had clinical symptoms, with cough in 84.1%, fever in 69.7%, and dyspnea in 36.2% of cases. In the LUS, 72.6% of participants had B-lines >2, 36.2% had coalescent B-lines, and 8.06% had subpleural consolidations. The median aeration score was 3 (2-7). The aeration score differed significantly regarding the presence of cough (P = .002), fever (P = .001), and dyspnea (P < .0001). The finding of subpleural consolidations in the LUS showed significant differences between participants with or without dyspnea (P < .0001). CONCLUSIONS: In healthcare professionals with COVID-19, LUS plays a key role in the characterization of lung involvement. Although B-lines are the most common ultrasound sign, subpleural consolidations are those that most impact the respiratory condition.

Effects of treatment with rituximab on microcirculation in patients with long-term systemic sclerosis.

OBJECTIVE: To investigate the effect of rituximab on microcirculation in long-term SSc. RESULTS: Four patients with diffuse SSc over 3 years of disease received rituximab cycles of two 1-g infusions every 6 months for 2 years. Videocapillaroscopy was performed at baseline, 12 months, and 24 months and semi-quantitative scoring of videocapillaroscopy abnormalities was performed and the microangiopathy evolution score (MES: range 0-9) was calculated. The mean disease duration was 5 years (range 3-15). On videocapillaroscopy, giant capillaries and hemorrhages remained stable from baseline to 24 months. Capillary loss, abnormally-shaped capillaries, and MES stabilized at 12 months and increased by 24.5% and 28% at 24 months. Rituximab improves microcirculation in long-term SSc. Stabilization and reduced progression of microcirculation abnormalities were achieved at 12 and 24 months, respectively.

Computed tomography trachea volumetry in patients with scleroderma: Association with clinical and functional findings.

BACKGROUND: In scleroderma, excessive collagen production can alter tracheal geometry, and computed tomography (CT) volumetry of this structure may aid in detecting possible abnormalities. The objectives of this study were to quantify the morphological abnormalities in the tracheas of ​​patients with scleroderma and to correlate these findings with data on clinical and pulmonary function. METHODS: This was a cross-sectional study in which 28 adults with scleroderma and 27 controls matched by age, gender and body mass index underwent chest CT with posterior segmentation and skeletonization of the images. In addition, all participants underwent pulmonary function tests and clinical evaluation, including the modified Rodnan skin score (mRSS). RESULTS: Most patients (71.4%) had interstitial lung disease on CT. Compared to controls, patients with scleroderma showed higher values ​​in the parameters measured by CT trachea volumetry, including area, eccentricity, major diameter, minor diameter, and tortuosity. The tracheal area and equivalent diameter were negatively correlated with the ratio between forced expiratory flow and forced inspiratory flow at 50% of forced vital capacity (FEF50%/FIF50%) (r = -0.44, p = 0.03 and r = -0.46, p = 0.02, respectively). The tracheal tortuosity was negatively correlated with peak expiratory flow (r = -0.51, p = 0.008). The mRSS showed a positive correlation with eccentricity (r = 0.62, p < 0.001) and tracheal tortuosity (r = 0.51, p = 0.007), while the presence of anti-topoisomerase I antibody (ATA) showed a positive correlation with tracheal tortuosity (r = 0.45, p = 0.03). CONCLUSIONS: In a sample composed predominantly of scleroderma patients with associated interstitial lung disease, there were abnormalities in tracheal geometry, including greater eccentricity, diameter and tortuosity. In these patients, abnormalities in the geometry of the trachea were associated with functional markers of obstruction. In addition, tracheal tortuosity was correlated with cutaneous involvement and the presence of ATA.

Eosinophilic granulomatosis with polyangiitis (formerly known as Churg-Strauss syndrome) as a differential diagnosis of hypereosinophilic syndromes.

Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, is a rare systemic disease situated between primary small vessel vasculitides associated with antineutrophil cytoplasmic antibodies (ANCAs) and hypereosinophilic syndromes (HES). Here, we present a case of EGPA in a 38-year-old male, with a previous diagnosis of asthma, who presented with fever, migratory lung infiltrates and systemic eosinophilia that was refractory to previous courses of antibiotics. This case highlights the importance of the primary care physician understanding the differential diagnosis of pulmonary eosinophilic syndromes.