Jorge Rojas-Serrano

Idiopathic Pulmonary Fibrosis and Emphysema: Decreased Survival Associated With Severe Pulmonary Arterial Hypertension

BACKGROUND It has been suggested that the presence of emphysema modifies the outcome of patients with idiopathic pulmonary fibrosis (IPF). In this article we compare clinical features, smoking history, pulmonary function, estimated systolic pulmonary artery pressure (eSPAP), and mortality in IPF with emphysema vs IPF without emphysematous changes. METHODS A cohort of 110 IPF patients was evaluated. Clinical data were collected from clinical charts. High-resolution CT (HRCT) scans were examined by an expert blinded to clinical data, and patients were classified into the following two groups: patients with IPF with emphysema; and patients with IPF without emphysema. The Kaplan-Meier method, log-rank test, and Cox regression model were used for statistical analyses. RESULTS The prevalence of emphysema in the IPF cohort was 28% (31 of 110 patients). IPF with emphysema was significantly associated with male gender (odds ratio [OR], 18; 95% confidence interval [CI], 2.7 to 773.7; p = 0.0003), and smoking (OR, 3.8; 95% CI, 1.36 to 11.6; p = 0.004). Patients with IPF and emphysema had a higher mean (+/- SD) decrease in oxygen saturation during rest and exercise (16.3 +/- 6.7% vs 13.5 +/- 4.6%, respectively; p = 0.04), a higher mean fibrosis HRCT scan score (1.75 +/- 0.36 vs 1.55 +/- 0.38, respectively; p = 0.015), a higher eSPAP (82 +/- 20 vs 57 +/- 15 mm Hg, respectively; p < 0.0001), and lower median survival time (25 vs 34 months, respectively; p = 0.01) than patients with IPF without emphysema. The Cox regression model showed that the two most important variables associated with mortality were FVC < 50% predicted (hazard ratio [HR], 2.6; 95% CI, 1.19 to 5.68; p = 0.016) and eSPAP >or= 75 mm Hg (HR, 2.25; 95% CI, 1.12 to 4.54; p = 0.022). CONCLUSIONS IPF patients with emphysema exhibited higher mortality compared with those with IPF without emphysema. This dire prognosis seems to be at least partially associated with the development of severe pulmonary arterial hypertension.

Interstitial lung disease related to rheumatoid arthritis: Evolution after treatment

OBJECTIVE To describe the evolution of lung function in a cohort of rheumatoid arthritis (RA) patients with interstitial lung disease (ILD) treated according to the medical judgment of attending physicians. METHODS Retrospective cohort of RA patients with ILD, defined by a restrictive pattern in lung function tests and evidence of ILD in high resolution computed tomography (HRCT). Patients had an assessment of lung function including spirometry, diffusing capacity for carbon monoxide (DLCO), and HRCT. At a minimum of 4 months of follow up, a second assessment of lung function was done. All patients received a high dose of prednisone (1 mg/kg/day) scheme for 6 weeks with a reduction scheme ending with a dose of 10 mg/day of prednisone at about 6-8 months of follow up. Methotrexate was used in 18/40 (45%) patients and leflunomide or azathioprine or both were indicated in 22/40 (55%). RESULTS Forty patients were identified. An indeterminate pattern with diffuse ground glass and reticulation images (50%) was the most prevalent pattern on HRCT scans. At a minimum of 4 months of follow up, an improvement in basal FVC values was observed (median (IQR)) 1.47 Lts. (0.99-1.91) vs 1.66 Lts. (1.37-2.1)), P<0.004. Patients with lower Kazerooni scores for fibrosis (<0.47) had a better improvement in the FVC values. CONCLUSIONS Patients with RA and ILD may have an improvement in the FVC after a treatment with high doses of corticosteroids and disease modifying antirheumatic drugs (DMARDs).

Antinucleosome antibodies may help predict development of systemic lupus erythematosus in patients with primary antiphospholipid syndrome.

Patients with primary antiphospholipid syndrome (PAPS) may evolve to systemic lupus erythematosus (SLE), even many years later. This makes differentiation between primary and secondary antiphospholipidsyndrome a difficult task. Studies in murine models of lupus have shown that the developmentof antinucleosome(anti-NCS) antibodiesmay occur from the early stages of life. We therefore hypothesizethat anti-NCS antibodies could help predict developmentof SLE in patients with PAPS. We studied anti-NCS antibodies in 18 PAPS patients (15 female, three male), followed for a mean of 11 years to evaluate the potential development of SLE. When PAPS was diagnosed, nine patients were positive for anti-NCS antibodies. Six of them developed clinical manifestationsof SLE. In contrast, none of the patients who were negative to anti-NCS antibodies developed it. These findings suggest that anti-NCS antibodiescould help predict which patients with PAPS may eventually develop SLE.

First acute gout attacks commonly precede features of the metabolic syndrome.

Objective:To determine in gout patients, the temporal relationship between the first gout attack and the diagnosis of metabolic syndrome (MS), its components and complications. Subjects and Methods:We included consecutive gout patients attending 2 Rheumatology Departments from Spain (Hospital Universitario Reina Sofía) and México (Hospital General de México). Variables included demographic, clinical, and biochemical data: Hypertension, hypertriglyceridemia, low high density lipoproteins (HDL), obesity, hyperglycemia or diabetes, MS (Adult Treatment Pane III criteria), ischemic heart disease (IHD), and chronic renal failure (CRF). Age and date (year) of the diagnosis of first acute gout attack and associated diseases were obtained. Results:Four hundred seven patients were included (96% men); mean age at onset, mean age at inclusion, and mean duration of the disease were 39.7 ± 13, 52.5 ± 13, and 13.7 ± 9.9 years, respectively. In 90%, the first attack of gout preceded the diagnosis of features of MS, MS itself or its complications (CRF and IHD), 9.8% had previous diagnosis of at least 1 associated disease. At the time of the inclusion (mean, 13.7 years after the first attack), 93% had at least 1 associated disease. The most common were hypertriglyceridemia, 63%; obesity, 54%; hypertension, 45.6%; MS, 40%; hyperglycemia, 37%; low HDL, 17%; diabetes, 15%; CRF, 17%; and IHD, 6.6%. Although patients from the 2 Rheumatology Departments had several demographic and clinical differences, in both groups most of the patients (70% Hospital Universitario Reina Sofía and 95% Hospital General de México) had no diagnosis of any associated disease previous to first bouts and at inclusion most of them had the diagnosis of at least 1 associated disease. Conclusions:First attacks of gout may precede the diagnosis of metabolic abnormalities and associated diseases, and provids a unique opportunity to diagnose, prevent, and/or retard long-term complications in these patients.

Rheumatoid arthritis-associated interstitial lung disease: Lung inflammation evaluated with high resolution computed tomography scan is correlated to rheumatoid arthritis disease activity

OBJECTIVE To describe the association between rheumatoid arthritis disease activity (RA) and interstitial lung damage (inflammation and fibrosis), in a group of patients with rheumatoid arthritis-associated interstitial lung disease (RA-ILD). METHODS A retrospective study of RA patients with interstitial lung disease (restrictive pattern in lung function tests and evidence of interstitial lung disease in high resolution computed tomography (HRCT)). Patients were evaluated to exclude other causes of pulmonary disease. RA disease activity was measured with the CDAI index. Interstitial lung inflammation and fibrosis were determined by Kazerooni scale. We compared Kazerooni ground-glass score with the nearest CDAI score to HRCT date scan of the first medical evaluation at our institution. In nine patients, we compared the first ground-glass score with a second one after treatment with DMARDs and corticosteroids. Spearmans rank correlation coefficient was used to evaluate association between RA disease activity and the Kazerooni ground-glass and fibrosis scores. RESULTS Thirty-four patients were included. A positive correlation between CDAI and ground-glass scores was found (rs=0.3767, P<0.028). Fibrosis and CDAI scores were not associated (rs=-0.0747, P<0.6745). After treatment, a downward tendency in the ground-glass score was observed (median [IQR]): (2.33 [2,3] vs. 2 [1.33-2.16]), P<0.056, along with a lesser CDAI score (27 [8-43] vs. 9 [5-12]), P<0.063. CONCLUSION There is a correlation between RA disease activity and ground-glass appearance in the HRCT of RA-ILD patients. These results suggest a positive association between RA disease activity and lung inflammation in RA-ILD.

Interstitial lung disease and myositis-specific and associated autoantibodies: Clinical manifestations, survival and the performance of the new ATS/ERS criteria for interstitial pneumonia with autoimmune features (IPAF)

OBJECTIVE to describe the clinical manifestations and survival of patients with ILD and myositis-specific and associated autoantibodies, and to evaluate the performance of the new ATS/ERS classification criteria for IPAF. PATIENTS AND METHODS Patients with ILD and positive in at least one of the following autoantibodies: anti-Jo-1, anti-Ej, anti-PL7, anti-PL 12, anti-PM/SCL 75 and anti-PM/SCL100 were included. Patients were separated into three groups according to their autoantibody profile: 1. Jo-1 positive patients, 2. Non-Jo-1 antisynthetase autoantibody positive patients, and 3. PM/SCL positive patients. Relevant clinical characteristics were registered. Patients were evaluated had they fulfilled Bohan and Peters criteria (BPC) for inflammatory myopathies. We evaluated the performance of the IPAF ATS/ERS proposal to classify as such the patients that did not fulfilled BPC, and evaluated whether IPAF patients had a worse survival that BPC patients. RESULTS Sixty-eight patients were included. Jo-1 was the most frequent autoantibody (65%), followed by non Jo1 anti-synthetase autoantibodies (31%). Non-Jo1 patients had lower Creatin Kinase serum levels at the baseline and less frequency of arthritis. Only 50% of patients fulfilled BPC. All patients not complying with BPC did comply with IPAF criteria. There was no difference in survival between IPAF and BPC patients. Anti Jo-1 positive was associated to survival and the extent of lung inflammation was associated to mortality. CONCLUSIONS Patients differ in clinical manifestations according to the autoantibody profile. All patients not complying with BPC did comply with the new IPAF criteria. There was no difference in survival between BPC and IPAF patients. Jo-1 patients had a better survival. Extent of lung inflammation was associate to mortality.

Rheumatoid arthritis-related interstitial lung disease (RA-ILD): methotrexate and the severity of lung disease are associated to prognosis

Interstitial lung disease (ILD) is a severe rheumatoid arthritis (RA) manifestation. The worst survival has been associated with usual interstitial pneumonia (UIP) definitive pattern in high-resolution chest tomography (HRCT) scans. Moreover, the use of methotrexate in RA-ILD is controversial. Our aim was to evaluate prognostic factors including methotrexate in an RA-ILD cohort and their association with survival. RA-ILD patients referred for medical evaluation and treatment at a single center were included. At the baseline, pulmonary function tests were carried out and a HRCT was obtained. A radiologist evaluated the ILD tomographic pattern and the extent of lung disease. Patients were considered as receiving methotrexate therapy if this drug was specifically prescribed for the treatment of RA-ILD at the beginning of follow up. Seventy-eight patients were included. UIP definite pattern in HRCT was not associated to worse survival. Variables associated with mortality reflected the severity of lung disease. Treatment with methotrexate was associated with survival (HR 0.13, 95% CI 0.02–0.64); older patients had worse prognosis (HR 1.04, 95% CI 1.003–1.09). After adjusting for confounding variables, methotrexate was strongly associated with survival. Methotrexate treatment during follow up was associated with survival. The severity of lung disease and not the tomographic pattern is associated with mortality; older patients had worse prognosis.

Menor incidencia de daño grave en órganos diana en pacientes mexicanos con esclerosis sistémica con afección cutánea difusa

OBJECTIVE To determine the cumulative incidence of severe organ involvement in Mexican patients with systemic sclerosis (SS) and diffuse scleroderma at 3 years from the onset of SS symptoms, and to compare itwith the cumulative incidence observed in a cohort of white patients with SS. PATIENTS AND METHOD Patients with SS and diffuse scleroderma were evaluated within the first 2 years from the onset of SS symptoms and were included. An estimation of the cumulative incidence of severe involvement to the skin, kidney, heart, lungs, and gastrointestinal track at 3 years from the onset of SS symptoms was carried out. This cumulative incidence was compared with that of white SS patients with diffuse scleroderma, using the one sample test for a binomial proportion. RESULTS Sixty-three patients were included. The cumulative incidence of severe involvement to the skin was 3.17% (2/63) (95% CI, 0.04%-11); kidney involvement in 4.17% (3/63) (95% CI, 0.99%-13.29%); heart involvement in 1.6% (1/63) (95% CI, 0.04%-8.5%); lung involvement in 11.11% (7/63) (95% IC, 4.5%-21.5%); and gastrointestinal involvement in 4.7% (3/63) (95% IC, 0.99%-13.3%). Mexican patients had a lower Reumatol Clin. 2008;4(1):3-7 3 02 ORIG 2582 (3-7).qxp 23/1/08 11:09 Página 4 Rojas-Serrano J et al. Incidencia de daño grave en pacientes mexicanos con esclerosis sistémica incidence of severe skin involvement (P=.0001), kidney involvement (P=.03) and heart involvement (P=.03) compared to white SS patients with diffuse scleroderma. CONCLUSIONS The cumulative incidence of severe organ involvement in SS Mexican patients with diffuse scleroderma was determined. The incidence of severe skin, kidney and heart involvement is lower than in white SS patients with diffuse scleroderma.

Hemorragia alveolar difusa: causas y desenlaces en un instituto de tercer nivel

OBJECTIVE To identify the most common causes of diffuse alveolar hemorrhage (DAH) and the evolution of cases during hospitalization. PATIENTS AND METHODS A review of cases diagnosed with DAH; the diagnoses were classified according to existing criteria and the progression of the cases was determined. RESULTS We identified 17 cases of DAH, with the leading cause being ANCA associated vasculitis (41% of cases), followed by cases secondary to drugs (18%). In 35% of the cases, there was a failure in identifying an etiology. Six patients died (35%), the only factor associated with mortality was male gender 5/6 vs 3/11, p=0.05. CONCLUSIONS The most frequent cause of alveolar hemorrhage was ANCA associated vasculitis. The mortality in DAH is about 35%, males seem to have a worse prognosis.

Clinical and Prognostic Factors Associated With Survival in Mexican Patients With Idiopathic Inflammatory Myopathies.

BackgroundFactors associated with survival in patients with idiopathic inflammatory myopathies are heterogeneous. ObjectiveThis study aimed to describe clinical and prognostic factors associated with survival in Mexican patients with idiopathic inflammatory myopathies. MethodsPatients with dermatomyositis (DM) and polymyositis (PM) seen at a tertiary care center from 1985 to 2012 were included. Demographic and clinical characteristics, comorbidities, treatment, and the time to death were recorded. Patients with juvenile DM were excluded. Univariate and multivariate analyses were performed to identify factors associated with mortality. ResultsA total of 264 patients with DM and 69 patients with PM were studied. Patients with DM had lower levels of creatine phosphokinase, less cumulative dose of prednisone, higher frequency of dysphagia, and no difference in frequency of interstitial lung disease compared with patients with PM. Patients with DM had lower survival during the first 4 years of disease (80%; 95% confidence interval [CI], 0.74–0.85 vs 89%; 95% CI, 0.78–0.95; P = 0.03 log-rank). Respiratory failure due to pulmonary infection was the main cause of death in patients with DM; miscellaneous causes were responsible for death in patients with PM. Muscular strength (hazard ratio [HR], 0.48; 95% CI, 0.27–0.83; P = 0.01), platelet count (HR, 0.98; 95% CI, 0.98–0.99; P = 0.002), as well as ever use of methotrexate (HR, 0.21; 95% CI, 0.07–0.65; P = 0.007) and azathioprine (HR, 0.21; 95% CI, 0.06–0.68; P = 0.009) were independent factors associated with mortality in patients with DM; in those with PM, only cancer was associated (HR, 8.0; 95% CI, 1.4–43.9; P = 0.01). ConclusionsPatients with DM had lower survival during the first 4 years of disease than patients with PM. Factors associated with mortality differed in both groups.