Vicente Fonollosa-Pla

Nailfold capillary microscopy in adults with inflammatory myopathy.

OBJECTIVES To study the presence and characteristics of nailfold capillary changes in a cohort of adult patients with inflammatory myopathies, determine correlations with disease activity and severity, and investigate any relationship between capillary findings and the immunological or clinical characteristics of the groups. METHODS Fifty-three consecutive adult patients followed in our outpatient clinic were evaluated using a Wild M3 stereomicroscope with an Intralux 5000 Volpi cold light lamp. A semiquantitative rating scale was used to score capillaroscopy changes. Disease activity and severity were assessed with the Myositis Disease Activity Assessment Tool and Myositis Damage Index, respectively. Associations between capillaroscopy findings and other factors were calculated with the chi(2) and Mann-Whitney U tests. Serum autoantibody profile was determined in all patients. RESULTS Twenty-three patients (43%) showed relevant capillaroscopy changes. No significant association was observed between the number of capillaroscopy alterations and the clinical or immunological groups, or disease duration. Disease activity and severity were both significantly associated with a larger number of capillaroscopy findings (P < 0.05). The combination of microhemorrhages and capillary enlargement was significantly more frequent in patients with dermatomyositis (OR, 8.9; 95% CI, 1.8-45.2), and a characteristic capillaroscopy pattern was associated with paraneoplastic myositis (OR, 14.7; 95% CI, 2.0-106.4). Interstitial lung disease significantly correlated with higher capillary score (OR, 3.7; 95% CI, 1.1-13.0). CONCLUSIONS Nailfold microcirculation as determined by semiquantitative simple capillaroscopy appears to provide useful information in patients with idiopathic inflammatory myopathy, contributing to an early diagnosis and identifying patients with a poor prognosis.

Age-related survival and clinical features in systemic sclerosis patients older or younger than 65 at diagnosis

OBJECTIVE To analyse the differences in SSc clinical features and survival in patients aged > or = 65 years compared with young SSc patients. METHODS Of a total of 319 SSc patients, we identified 67 (21%) patients aged >65 years. Demographical data such as SSc subsets, the cutaneous complaint, internal organ involvement and the causes of morbidity and mortality were collected. Results of the elderly and young patients were compared. RESULTS There were 61 (91%) women and 6 (9%) men aged > or = 65 years. The limited SSc (lSSc) subset was more prevalent in elderly than in young patients (74.6 vs 54%, P = 0.002). Pulmonary disease (86.6% in elderly vs 73.8% in young patients, P = 0.034) and cardiac involvement (70.1% in elderly vs 49.6% in young patients, P = 0.004) were significantly more prevalent in elderly patients. In contrast, signs of oesophageal involvement (43.3% in elderly vs 57.5% in young patients, P = 0.040) were less frequent in aged patients. In addition, pulmonary and heart disease appeared significantly earlier after the diagnosis in patients aged > or = 65 years. Mortality was significantly higher in elderly than in young patients (35.8 vs 19%, P = 0.005), but when standardized mortality ratios (SMRs) were analysed, there was no significant mortality increase in the elderly. CONCLUSION In elderly patients, the lSSc subset is more prevalent than the diffuse. Pulmonary and cardiac involvement are more prevalent in aged patients and appears sooner after the disease diagnosis. SSc is clearly related to increased mortality, although it is not significant in the elderly group.

Registry of the Spanish Network for Systemic Sclerosis: Survival, Prognostic Factors, and Causes of Death.

AbstractSystemic sclerosis (SSc) is a rare, multisystem disease showing a large individual variability in disease progression and prognosis. In the present study, we assess survival, causes of death, and risk factors of mortality in a large series of Spanish SSc patients. Consecutive SSc patients fulfilling criteria of the classification by LeRoy were recruited in the survey. Kaplan–Meier and Cox proportional-hazards models were used to analyze survival and to identify predictors of mortality. Among 879 consecutive patients, 138 (15.7%) deaths were registered. Seventy-six out of 138 (55%) deceased patients were due to causes attributed to SSc, and pulmonary hypertension (PH) was the leading cause in 23 (16.6%) patients. Survival rates were 96%, 93%, 83%, and 73% at 5, 10, 20, and 30 years after the first symptom, respectively. Survival rates for diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc were 91%, 86%, 64%, and 39%; and 97%, 95%, 85%, and 81% at 5, 10, 20, and 30 years, respectively (log-rank: 67.63, P < 0.0001). The dcSSc subset, male sex, age at disease onset older than 65 years, digital ulcers, interstitial lung disease (ILD), PH, heart involvement, scleroderma renal crisis (SRC), presence of antitopoisomerase I and absence of anticentromere antibodies, and active capillaroscopic pattern showed reduced survival rate. In a multivariate analysis, older age at disease onset, dcSSc, ILD, PH, and SRC were independent risk factors for mortality. In the present study involving a large cohort of SSc patients, a high prevalence of disease-related causes of death was demonstrated. Older age at disease onset, dcSSc, ILD, PH, and SRC were identified as independent prognostic factors.

Tratamiento del fenómeno de Raynaud

Raynauds phenomenon (RP) is a clinical picture characterized by the presence of recurrent episodes of vasospasm that is precipitated by cold or other stimuli and especially affects the fingers and/or toes. It may be primary or idiopathic or secondary and be due to many causes, among which connective diseases, and specifically scleroderma, stand out. Primary RP does not generally require drug treatment, only requiring general measures. Calcium channel antagonists continue to be the drug of first choice. However, when RP is severe and is accompanied by digital ulcers/tissue necrosis, the therapeutic regime must be individualized and combinations should be established with difference drugs such as prostanoids, bosentan, sildenafil, antiaggregants/anticoagulants, antibiotics and analgesics.

Valor pronóstico del pH en el condensado de aire exhalado y de la fracción exhalada de óxido nítrico en la enfermedad pulmonar intersticial asociada a esclerosis sistémica

INTRODUCTION Interstitial lung disease (ILD) is one of the major causes of death in systemic sclerosis (SSc). This study investigated exhaled breath (EB) and exhaled breath condensate (EBC) biomarkers in patients with SSc and analyzed their role as a prognostic tool in SSc-related ILD. METHODS Fraction exhaled nitric oxide (FeNO) and exhaled carbon monoxide (eCO) measured in EB, together with pH, nitrite, nitrate and interleukin-6 levels measured in EBC were prospectively analyzed in 35 patients with SSc. Twelve patients had established ILD by chest high-resolution computed tomography (HRCT), and 23 patients showed no evidence of ILD. EB and EBC biomarkers were determined at inclusion, and pulmonary function tests were annually performed during 4 years of follow-up. RESULTS No differences at baseline biomarkers levels were found between groups. In all patients studied, low EBC pH levels were associated with a decreased diffusing capacity for carbon monoxide (DLCO) during follow-up. Low FeNO levels were correlated with lower forced vital capacity (FVC) at baseline, 4years of follow-up and with a decrease in FVC and DLCO during monitoring. Among ILD patients, high eCO levels were correlated with lower baseline FVC. In the global cohort, a worse progression-free survival was identified in patients with EBC pH values lower than 7.88 and FeNO levels lower than 10.75ppb (Log Rank P=.03 and P<.01, respectively). CONCLUSIONS EB and EBC could help to detect patients likely to present a deterioration on lung function during follow up.

Implicación pronóstica de las manifestaciones clínicas extrahepáticas, autoinmunidad y capilaroscopia ungueal microscópica en pacientes con cirrosis biliar primaria

BACKGROUND AND OBJECTIVES Primary biliary cirrhosis (PBC) is associated to any systemic autoimmune disease (SAD), in particular systemic sclerosis (SSc). To investigate the prevalence of SAD in a cohort of patients with PBC, specifically the prevalence of SSc and its clinical subtypes, and determining the clinical and biological profile of patients with associated PBC and SSc. METHODS Observational study of 62 patients with PBC following a protocol that included an anamnesis and physical examination to detect the presence of SAD as well as a nailfold capillaroscopy and an immunological study with specific SSc autoantibodies. A comparative analysis was conducted between patients with isolated PBC and patients with PBC and an associated SAD. RESULTS SAD was associated to PBC in 22 patients (35,4%), and SSc was the most frequent illness, identified in 13 cases (21%). Five patients (8%) without previous diagnosis of SAD fulfilled pre-scleroderma criteria, according to LeRoy and Medsger criteria. The presence of anticentromere antibodies (54,5% vs. 5%, P<.001) was the unique immunological determination identified more frequently in patients with PBC-SAD. The SSc suggestive capillary pattern was visualized in 11 patients (20,4%), mainly the slow pattern. No factors associated with greater morbi-mortality were identified in the PBC-SAD group. CONCLUSIONS It does exist a subgroup of patients with PBC and clinical-biological features suggestive of an SAD, which advise a protocolized study to detect early the association to an SAD.

FRI0442 Prognostic Role of Exhaled Breath Condensate in Patients with Pulmonary Involvement Associated to Systemic Sclerosis

Background Lung involvement due to interstitial lung disease (ILD) and/or pulmonary arterial hypertension (PAH) is the most important cause of death in systemic sclerosis (SSc). Objectives To assess the clinical usefulness of exhaled breath and exhaled breath condensate (EBC) biomarkers in the study of scleroderma pulmonary involvement (PI), and their correlation with disease progression. Methods Fraction exhaled nitric oxide (FeNO) and exhaled carbon monoxide (eCO) measured in exhaled breath and pH, nitrite, nitrate and interleukin-6 in EBC were prospectively collected from 35 patients with SSc. Fourteen had established PI as presence of ILD or PAH, and 21 patients with no lung involvement. Pulmonary function tests (PFT) and clinical approaches were performed annually during 4 years of follow-up. A progression-free survival (PFS) analysis was performed defining end point as a decrease from baseline FVC of at least 10%, or a 15% diminution in DLCO, or death during follow-up. Results There were no statistical differences in demographic data and the baseline characteristics between both groups. Median (interquartile range, IQR) age was 59.0 (42.0 to 68.0) years. Limited SSc was the most common cutaneous subset (28, 80%). The median of pulmonary duration disease was 1.7 years in PI group. Regarding lung function data at baseline, lower median FVC % and DLCO % values were identified in the PI group (76.5 vs. 94.2, p<0.01) and (45.6 vs. 71.7, p<0.001), respectively. Lower baseline pH levels were found in PI group (7.5 vs 8.0, p=0.04), with no other biomarker differences. Respecting the correlations between EBC biomarkers and PFTs during monitoring, lower pH levels were associated with lower DLCO % at 4 years of follow-up (r=0.45, p=0.01). Reduced FeNO levels were correlated with low FVC % at baseline (r=0.41, p=0.03), 4 years of follow-up (r=0.46, p=0.02) and with a decrease of FVC % (r=0.65, p<0.001) and DCLO % during monitoring (r=0.50, p=0.01). Among the PI patients, high eCO measurements were related to low baseline FVC % (r=0.69, p<0.01), and high IL-6 levels were correlated with low DLCO % at 4 years of follow-up (r= -0.64, p=0.03). The progression-free survival analysis identified that a pH equal or lower than 7.85 showed a decrease in lung function parameters or death during follow-up (log Rank p=0.03). FeNO levels equal or lower than 11.0 ppb were related to worse PFS (log Rank p<0.01) (figure). Conclusions The pH and FeNO levels are implicated in the pathophysiology of the scleroderma lung. Exhaled breath and EBC may reflect the pulmonary inflammation with a non-invasive test, in which the biomarkers are correlated with a different outcome. Disclosure of Interest None declared