The SLICC-FI Predicts Damage Accrual in SLE Patients. Data From the Almenara Lupus Cohort.

OBJECTIVE: To evaluate the Systemic Lupus International Collaborating Clinics Frailty Index (SLICC-FI) as a predictor of damage accrual in a primarily Mestizo SLE patient cohort. METHODS: Patients from a single-center prevalent cohort were included. Damage accrual was defined as the increase in the SLICC/American College of Rheumatology (ACR) damage index (SDI) scores between the baseline and the last visits. The SLICC-FI was measured at baseline. Univariable and multivariable Cox regression models were performed to determine the association between the baseline SLICC-FI (per 0.05 increase) and the increase in the SDI, adjusted for possible confounders. Alternative analyses using negative binomial regression models including the difference between the last and the first SDI as outcome were performed. RESULTS: Of the 265 patients included, 248 (93.6%) were female with mean (SD) age of 35.1 (13.6) years at diagnosis. At baseline, mean (SD) SLE disease duration was 7.3 (6.5) years, SDI was 1.0 (1.2) and the SLICC-FI was 0.22 (0.05). After a mean (SD) of 5.2 (2.2) years of follow-up, the SDI increased in 126 (47.5%) patients, and the final mean (SD) SDI score was 1.7 (1.7). Higher SLICC-FI scores at baseline predicted greater damage accrual in the univariable analysis [Hazard Ratio (HR) =1.38, (CI95% 1.16-1.65); p < 0.001] and in the multivariable model, after adjustment for possible confounders [HR = 1.30 (CI95% 1.02-1.66); p = 0.033]. CONCLUSION: SLICC-FI predicts the occurrence of damage accrual in a prevalent SLE Latin-American cohort with short or long disease duration, supporting the relevance of this index in the evaluation of SLE patients.

Metabolic syndrome predicts new damage in systemic lupus erythematosus patients: Data from the Almenara Lupus Cohort.

OBJECTIVES: This study aims to determine whether the MetS predicts damage accrual in SLE patients. METHODS: This longitudinal study was conducted in a cohort of consecutive SLE patients seen since 2012 at one single Peruvian institution. Patients had a baseline visit and then follow-up visits every 6 months. Patients with ≥ 2 visits were included. Evaluations included interview, medical records review, physical examination, and laboratory tests. Damage accrual was ascertained with the SLICC/ACR damage index (SDI) and disease activity with the SLEDAI-2K. Univariable and multivariable Cox-regression survival models were carried out to determine the risk of developing new damage. The multivariable model was adjusted for age at diagnosis; disease duration; socioeconomic status; SLEDAI; baseline SDI; the Charlson Comorbidity Index; daily dose; and time of exposure of prednisone (PDN), antimalarials, and immunosuppressive drugs. RESULTS: Two hundred and forty-nine patients were evaluated; 232 of them were women (93.2%). Their mean (SD) age at diagnosis was 35.8 (13.1) years; nearly all patients were Mestizo. Disease duration was 7.4 (6.6) years. The SLEDAI-2K was 5.2 (4.3) and the SDI, 0.9 (1.3). One hundred and eight patients (43.4%) had MetS at baseline. During follow-up, 116 (46.6%) patients accrued at least one new point in the SDI damage index. In multivariable analyses, the presence of MetS was a predictor of the development of new damage (HR: 1.54 (1.05-2.26); p < 0.029). CONCLUSIONS: The presence of MetS predicts the development of new damage in SLE patients, despite other well-known risk factors for such occurrence.

Performance of the 2017 American College of Rheumatology/European League Against Rheumatism Provisional Classification Criteria for Antineutrophil Cytoplasmic Antibody-Associated Vasculitis in a Peruvian Tertiary Care Center.

AIM: To validate the new classification criteria for antineutrophil cytoplasmic antibody-associated vasculitis in a real-life Peruvian cohort of antineutrophil cytoplasmic antibody-associated vasculitis patients. METHODS: We reviewed medical records from a Peruvian tertiary care center from January 1990 to December 2019. Antineutrophil cytoplasmic antibody-associated vasculitis was diagnosed based on the 1990 American College of Rheumatology (ACR) criteria, the 2012 Chapel Hill Consensus Conference definitions, the European Medicines Agency (EMEA) algorithm, and the clinical acumen of the treating rheumatologists. We classified all patients using the "former criteria" (the 1990 ACR criteria for granulomatosis with polyangiitis [GPA] and eosinophilic GPA [EGPA] and the 1994 Chapel Hill Consensus Conference definition for microscopic polyangiitis [MPA]), the EMEA algorithm, and the "new criteria" (the 2017 ACR/European League Against Rheumatism Provisional Criteria). The level of agreement (using Cohen κ) was calculated using the clinical diagnosis as the criterion standard. RESULTS: We identified 212 patients, 12 of whom were excluded. One hundred fifty-four (77%) had MPA, 41 (20.5%) GPA, and 5 (2.5%) EGPA. The new criteria performed well for MPA (κ = 0.713) and EGPA (κ = 0.659), whereas the EMEA algorithm performed well for GPA (κ = 0.938). In the overall population, the new criteria showed better agreement (κ = 0.653) than the EMEA algorithm (κ = 0.506) and the former criteria (κ = 0.305). CONCLUSIONS: The 2017 ACR/European League Against Rheumatism Provisional Criteria showed better agreement for the clinical diagnosis of all the patients overall and had the best performance for MPA and EGPA. The EMEA algorithm had the best performance for GPA.

Survival in ANCA-Associated Vasculitides in a Peruvian Center: 28 Years of Experience.

AIM: The aim of this study was to identify demographic and clinical risk factors for mortality in patients with antineutrophil cytoplasmic antibodies-associated vasculitides (AAVs) in a Peruvian tertiary referral hospital. METHODS: Medical records of patients with AAV according to classification criteria or diagnosed by an experienced rheumatologist, covering the period between January 1990 and December 2018, were reviewed. Granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis, and renal-limited vasculitis were included. Potential predictors of mortality were demographic factors, clinical manifestations, antineutrophil cytoplasmic antibodies status, diagnosis, disease categorization, the 2009 Five Factor Score (FFS), and treatment. Cox regression models were used to determine the risk factors for mortality. Univariable and multivariable analyses using a backward selection method were performed. RESULTS: One hundred ninety-six patients were included; female-to-male ratio was 2:1. The median (interquartile range) age at diagnosis and follow-up were 60.0 (51.0-68.0) and 4.8 (1.3-11.6) years, respectively. One hundred forty-eight patients (75.5%) had microscopic polyangiitis, 37 (18.9%) granulomatosis with polyangiitis, 5 (2.6%) eosinophilic granulomatosis with polyangiitis, and 6 (3.0%) renal-limited vasculitis. Overall survival rates at 1, 5, and 10 years were 83.4%, 68.2%, and 51.7%, respectively. Ocular involvement was protective (hazards ratio [HR], 0.36; 95% confidence interval [CI], 0.17-0.74; p = 0.006), whereas renal (HR, 2.09; 95% CI, 1.33-3.28; p = 0.001) and lung involvement (HR, 2.07; 95% CI, 1.31-3.28; p = 0.002) and the 2009 FFSs were predictive of mortality (2009 FFS = 1: HR, 2.46; 95% CI, 1.50-4.04; p < 0.001; 2009 FFS = 2: HR, 3.07; 95% CI, 1.54-6.10; p = 0.001; 2009 FFS = 3: HR, 13.29; 95% CI, 3.69-47.88; p < 0.001). CONCLUSIONS: Ocular involvement was protective, whereas 2009 FFS ≥ 1 and renal and lung involvement were predictive factors of mortality in Peruvian AAV patients.

Demographic and Clinical Features of ANCA-Associated Vasculitides in a Peruvian Tertiary Center.

AIM: The aim of this study was to identify the demographic and clinical features of patients with ANCA-associated vasculitides (AAVs) in a Peruvian tertiary referral hospital. METHODS: Medical records of patients with AAV according to classification criteria or diagnosed by an experienced rheumatologist, and covering the period between January 1990 and December 2019, were reviewed. Granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA), and renal-limited vasculitis (RLV) were included. Demographic factors (age at diagnosis, sex), disease duration, clinical manifestations (per organ involvement), creatinine level at diagnosis (milligram per deciliter), ANCA status, diagnosis, 2009 Five Factor Score, disease categorization, and treatment were recorded. RESULTS: Two hundred twelve patients were included. Their female-to-male ratio was 1.9:1 (139 [65.6%]/73 [34.4%]), and their mean (SD) age at diagnosis was 59.2 (12.5) years. One hundred fifty-eight patients (74.5%) had MPA, 42 (19.8%) GPA, 7 (3.3%) RLV, and 5 (2.4%) EGPA. Neurological, lung, and renal involvements were the most frequently affected systems. Myeloperoxidase preferentially occurred in MPA (82.5%), whereas proteinase 3 did occur in GPA (79.5%). Microscopic polyangiitis patients were older (61.1 [11.5] years). Female sex predominated in MPA and RLV (2.4:1 and 6:1, respectively), but the opposite was the case for EGPA (1:4). Ear-nose-throat and ocular involvement were more frequent in GPA (both p's < 0.001), and neurological and cardiovascular involvement were more frequent in EGPA (p < 0.001 and p = 0.002, respectively). CONCLUSIONS: This is one of the largest series of AAV patients in Latin America. Overall, female sex predominated. Microscopic polyangiitis was the most frequent AAV, and myeloperoxidase-ANCA was the most frequent antibody in Peruvian AAV population.

Age at diagnosis and health-related quality of life are associated with fatigue in systemic lupus erythematosus patients: Data from the Almenara Lupus Cohort.

OBJECTIVE: To define the factors associated with fatigue in Mestizo patients with Systemic Lupus Erythematosus (SLE). METHODS: This is a cross-sectional study of SLE patients from a single center cohort. Visits were performed every six months. For these analyses, the first visit between October 2017 and December 2018 was included. Demographic and clinical characteristics as well as treatment were recorded at every visit. Fatigue was ascertained with the Functional Assessment of Chronic Illness Therapy-Fatigue (FACIT-FT), Health-Related Quality of Life (HRQoL) with the LupusQoL, disease activity with the Systemic Lupus Erythematosus Disease Activity Index -2 K (SLEDAI-2K), and damage with the Systemic Lupus International Collaborating Clinics (SLICC)/American College of Rheumatology damage index (SDI). Prednisone use was recorded as current daily dose. Immunosuppressive drugs and antimalarial use were recorded as current, past or never. Univariable and multivariable analyses were performed using linear regression models. For the multivariable analyses, model selection followed a backward elimination procedure. RESULTS: Two hundred and twenty-six patients were evaluated. The mean (SD) age at diagnosis was 35.6 (13.1) years, 211 (93.4%) were female; and disease duration was 11.0 (7.3) years. The mean SLEDAI and SDI were 2.4 (3.5) and 1.3 (1.5), respectively. The mean FACIT-FT was 33.1 (10.8). On the multivariable analysis, age at diagnosis and some domains of HRQoL (physical health, emotional health and fatigue) remained associated. CONCLUSIONS: Age at diagnosis is negatively associated with fatigue whereas HRQoL domains like physical health, emotional health and fatigue are positively associated with fatigue.

Better Health-Related Quality of Life in Systemic Lupus Erythematosus Predicted by Low Disease Activity State/Remission: Data From the Peruvian Almenara Lupus Cohort.

OBJECTIVE: To determine if low disease activity state (LDAS)/remission predicts a better health-related quality of life (HRQoL). METHODS: Patients with systemic lupus erythematosus from a single center and having completed at least 2 visits were included. Visits were performed every 6 months. HRQoL was measured with the LupusQoL questionnaire. The definition of remission included a Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) score of 0, prednisone daily dosage of ≤5 mg/day, and immunosuppressive drugs on maintenance dose. LDAS was defined as a SLEDAI-2K score of ≤4, prednisone daily dosage of ≤7.5 mg/day, and immunosuppressive drugs as maintenance therapy. For these analyses, remission and LDAS were combined as one variable. Generalized estimating equations were calculated, using as the outcome 1 of each of the 8 components of the LupusQoL questionnaire in the subsequent visit and the activity state in the previous visit. Multivariable models were adjusted for possible confounders. RESULTS: A total of 243 patients were included. During the follow-up, 590 visits (61.6%) were categorized as LDAS/remission. LDAS/remission predicted a better HRQoL in the components of physical health (B = 4.17 [95% confidence interval (95% CI) 1.20, 7.14]; P = 0.006), pain (B = 6.47 [95% CI 3.18, 9.76]; P < 0.001), planning (B = 4.97 [95% CI 1.43, 8.52]; P = 0.006), burden to others (B = 4.12 [95% CI 0.24, 8.01]; P = 0.037], emotional health (B = 4.50 [95% CI 1.56, 7.44]; P = 0.003), and fatigue (B = 3.25 [95% CI 0.04, 6.47]; P = 0.048). CONCLUSION: Being in LDAS/remission predicts a better HRQoL, especially in the components of physical health, pain, planning, burden to others, emotional health, and fatigue.

Correction to: Homocysteine levels are independently associated with damage accrual in systemic lupus erythematosus patients from a Latin-American cohort.

The affiliations of Manuel F. Ugarte-Gil, one of the author of the above article has a discrepancy between the online version of the publisher's internet portal ( Springerlink.com ) and the online pdf version.

Homocysteine levels are independently associated with damage accrual in systemic lupus erythematosus patients from a Latin-American cohort.

OBJECTIVE: To determine the impact of homocysteine levels on damage accrual in systemic lupus erythematosus (SLE) patients. METHODS: This longitudinal study was conducted in consecutive patients seen every 6 months at our Rheumatology Department since 2012. Patients with available homocysteine levels and who had at least one subsequent visit were included. Univariable and multivariable Cox regression models were done to determine if homocysteine levels were predictive of damage accrual as per the SLICC Damage Index (SDI). The multivariable model was adjusted for pertinent variables (age at diagnosis, gender, socioeconomic status, disease duration, disease activity (SLEDAI), Framingham score, antimalarial and immunosuppressive drug use, average daily dose, and exposure time to prednisone (PDN)). RESULTS: One hundred forty-five patients were included; their mean (SD) age at diagnosis was 43.70 (12.09) years, 136 (93.8%) were female, and nearly all were Mestizo. At baseline, disease duration was 7.55 (6.73) years; patients were followed for 3.54 (1.27) years. The SLEDAI was 5.60 (4.34), and the SDI 0.97 (1.35). The average daily PDN dose was 7.30 (5.78) mg/day and the time of PDN exposure was 7.36 (6.73) years. Mean homocysteine levels were 10.07 (3.71) µmol/L. The highest tertile of homocysteine levels predicted new damage accrual in the univariable and multivariable models [HR 1.78 (95% CI, 1.042-3.039); p = 0.035 and HR 2.045 (95% CI, 1.077-3.883); p = 0.029, respectively]. Increased levels (> 15 µmol/L) were found in 12 (8.3%) patients; 75 (51.7%) patients increased ≥ 1 SDI point. CONCLUSION: In SLE patients, homocysteine levels predicted damage accrual independently of other well-known risk factors for such occurrence.

Factors associated with health-related quality of life in Peruvian patients with systemic lupus erythematosus.

Objective In this paper, we aim to define factors associated with health-related quality of life (HRQoL) in Mestizo patients with systemic lupus erythematosus (SLE). Methods We evaluated patients with SLE from Peru's two largest hospitals between October 2012 and July 2015 to ascertain HRQoL. Using a standard protocol, we incorporated demographic characteristics, clinical manifestations and treatment in our analysis. HRQoL was measured with the LupusQoL, disease activity was ascertained with the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI), and damage was appraised with the Systemic Lupus International Collaborating Clinics (SLICC)/American College of Rheumatology (ACR) damage index (SDI). The associations between the LupusQoL and these variables were examined using linear regression models. Model selection was based on backward elimination. Results A total of 277 patients fit the inclusion criterion. Of these, 254 (91.7%) were female, the median (interquartile range, IQR) age at diagnosis was 41.5 (33.8-51.8) years, disease duration was 6.5 (2.7-11.3) years. The HRQoL domains most affected were the following: burden to others, fatigue, and intimate relationships. Through multivariate analysis, we determined that older age at diagnosis, higher disease activity, damage, and immunosuppressive drug use were negatively associated with HRQoL. Further, we found that higher socioeconomic status, disease duration, and antimalarial use were positively associated with HRQoL. Conclusion Age at diagnosis, disease activity, damage, and use of immunosuppressive drugs were negatively associated with HRQoL; high socioeconomic status, disease duration, and use of antimalarials were positively associated with HRQoL.

Serum uric acid levels contribute to new renal damage in systemic lupus erythematosus patients.

This study aims to determine whether uric acid levels contribute to new renal damage in systemic lupus erythematosus (SLE) patients. This prospective study was conducted in consecutive patients seen since 2012. Patients had a baseline visit and follow-up visits every 6 months. Patients with ≥2 visits were included; those with end-stage renal disease (regardless of dialysis or transplantation) were excluded. Renal damage was ascertained using the SLICC/ACR damage index (SDI). Univariable and multivariable Cox-regression models were performed to determine the risk of new renal damage. Uric acid was included as a continuous and dichotomous (per receiving operating characteristic curve) variable. Multivariable models were adjusted for age at diagnosis, disease duration, socioeconomic status, SLEDAI, SDI, serum creatinine, baseline use of prednisone, antimalarials, and immunosuppressive drugs. One hundred and eighty-six patients were evaluated; their mean (SD) age at diagnosis was 36.8 (13.7) years; nearly all patients were mestizo. Disease duration was 7.7 (6.8) years. Follow-up time was 2.3 (1.1) years. The SLEDAI was 5.2 (4.3) and the SDI 0.8 (1.1). Uric acid levels were 4.5 (1.3) mg/dl. During follow-up, 16 (8.6%) patients developed at least one new point in the renal domain of the SDI. In multivariable analyses, uric acid levels (continuous and dichotomous) at baseline predicted the development of new renal damage (HR 3.21 (1.39-7.42), p 0.006; HR 18.28 (2.80-119.48), p 0.002; respectively). Higher uric acid levels contribute to the development of new renal damage in SLE patients independent of other well-known risk factors for such occurrence.

Garre's sclerosing osteomyelitis caused by salmonella group D in a patient with systemic lupus erythematosus: an unusual complication.

We report the case of a 35-year-old male, who was diagnosed with systemic lupus erythematosus (SLE) in 2010 based on the presence of articular, serous, renal, immune, and hematologic involvement. He also had secondary antiphospholipid syndrome (APS). He was treated with prednisone 10 mg per day, hydroxychloroquine 200 mg per day, methotrexate 12.5 mg per week, leflunomide 20 mg per day, and oral anticoagulation previous to the present event. He presented to emergency room with a 7 day disease duration characterized by pain in the left thigh, which increased with physical activity, resulting in claudication; he also had malaise and fever. The X-ray films showed periostitis of the lower half of the left femur with bone marrow narrowing; the scintigraphy showed marked increased uptake in the middle and distal thirds of the left femur, and magnetic resonance imaging (MRI) showed thickening and hyperintensity of the cortex of the diaphysis and distal epiphysis of the femur and endosteal irregularity. Empirical treatment was started with vancomycin for 3 weeks. Femur biopsy and cultures were performed, isolating Salmonella spp. group "D" Vi (-); treatment with cotrimoxazole and ceftazidime for 4 weeks followed by doxycycline and cotrimoxazole for 4 months were given with a favorable functional outcome. This is an unusual case of a young adult with Garre's sclerosing osteomyelitis associated to SLE and caused by salmonella. The literature is reviewed and the clinical conditions predisposing to this infection are discussed, particularly in patients with SLE.

Circulating naive and memory CD4+ T cells and metabolic syndrome in patients with systemic lupus erythematosus: data from a primarily Mestizo population.

OBJECTIVE: The aim of this study was to determine whether the proportions of naive and memory CD4(+) T cell are independently associated with the metabolic syndrome (MetS) in patients with SLE. METHODS: This cross-sectional study was conducted in SLE patients seen at our rheumatology department between September 2013 and April 2014. CD4(+) T cell subpopulations were examined by flow cytometry. The association of MetS and CD4(+) T cell subpopulations was examined by Mann-Whitney U-test and by multivariable analysis, adjusting for all possible confounding variables. RESULTS: One hundred and seventeen patients were evaluated. Their mean age was 44.6 years (S.D. 12.6), 109 (93.2%) were female and all patients were Mestizo (mixed Caucasian and Amerindian ancestry). Fifty-two patients (44.4%) presented with MetS. Disease duration was 7.6 years (S.D. 6.8). The percentage of naive CD4(+) T cells was 25.0 (S.D. 12.7) and memory CD4(+) T cells was 66.7 (S.D. 13.2) and the memory:naive CD4(+) T cell ratio was 4.3 (S.D. 5.6). In multivariable analysis, the percentage of naive CD4(+) T cells was negatively associated with the presence of MetS [odds ratio (OR) 0.959 (95% CI 0.923, 0.997), P = 0.033], whereas the percentage of memory CD4(+)T cells and the memory:naive CD4(+) T cell ratio were positively associated with its presence [OR 1.040 (95% CI 1.003, 1.078), P = 0.031 and OR 1.238 (95% CI 1.041, 1.472), P = 0.016, respectively]. CONCLUSION: In the SLE patients studied, a lower percentage of naive CD4(+) T cells, a higher percentage of memory CD4(+) T cells and the memory:naive CD4(+) T cell ratio were independently associated with the presence of MetS. This association could reflect the impact of immunosenescence among SLE patients with cardiovascular morbidity.

The number of flares patients experience impacts on damage accrual in systemic lupus erythematosus: data from a multiethnic Latin American cohort.

PURPOSE: To determine the association between the number of flares systemic lupus erythematosus (SLE) patients experience and damage accrual, independently of other known risk factors. METHODS: SLE patients (34 centres, nine Latin American countries) with a recent diagnosis (≤2 years) and ≥3 evaluations were studied. Disease activity was ascertained with the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and damage with the SLICC/ACR Damage Index (SDI). Flare was defined as an increase ≥4 points in the SLEDAI between two study visits. An ambidirectional case- crossover design was used to determine the association between the number of flares and damage accrual. RESULTS: 901 patients were eligible for the study; 500 of them (55.5%) experienced at least one flare, being the mean number of flares 0.9 (SD: 1.0). 574 intervals from 251 patients were included in the case-crossover design since they have case and control intervals, whereas, the remaining patients did not. Their mean age at diagnosis was 27.9 years (SD: 11.1), 213 (84.9%) were women. The mean baseline SDI and SLEDAI were 1.3 (1.3) and 13.6 (8.1), respectively. Other features were comparable to those of the entire sample. After adjusting for possible confounding variables, the number of flares, regardless of their severity, was associated with damage accrual (SDI) OR 2.05, 95% CI 1.43 to 2.94, p<0.001 (OR 2.62, 95% CI 1.31 to 5.24, p=0.006 for severe and OR 1.91, 95% CI 1.28 to 2.83, p=0.001 for mild-moderate). CONCLUSIONS: The number of flares patients experience, regardless of their severity, increases the risk of damage accrual, independently of other known risk factors.

Tratamiento con ciclofosfamida en enfermedad pulmonar intersticial secundaria a enfermedades difusas del tejido conectivo en el Hospital Nacional Guillermo Almenara IrIgoyen-EsSalud. 1998-2000 / Treatment with cyiclophosphamide in pulmonary intermediate secondary disease to diffuse diseases of the connective fabric in the National Hospital Guillermo Almenara Irigoyen-EsSalud. 1998-2000

Objetivo: Evaluar la respuesta al tratamiento con ciclofosfamida endovenosa (CFA EV) intermitente en pacientes con enfermedad pulmonar intersticial difusa (EPID) secundaria a enfermedad difusa de tejido conectivo (EDTC) atendidos en el Hospital Nacional Guillermo Almenara Irigoyen de enero 1998 a diciembre 2000. Métodos: Se realizó el análisis y revisión sistemática de los casos con diagnóstico de EPID secundaria a EDTC (Lupus Eritematoso Sistémico (LES), Dermatomiositis (DM) y Esclerosis Sistémica (ES) seguidos en el servicio de Reumatología del HNGAI entre enero de 1998 a diciembre 2000. Estos pacientes cumplieron con los criterios de inclusión quienes recibieron como protocolo de tratamiento Ciclofosfamida endovenosa intermitente durante 1 año, que cumplieron con los criterios de inclusión planteados. Se evaluaron clínica, gasometría, función respiratoria, lavado bronquioalveolar, radiografía simple y TAC pulmonar helicoidal de alta resolución al inicio, 6 meses y 12 meses del tratamiento. Resultados: Se evaluaron 5 pacientes seleccionados, 3 mujeres, tuvieron diagnóstico LES 1 con DM y un paciente con ES con promedio de edad 52,6 años (rango 33 a 61 años). El tiempo de enfermedad de la EDTC al diagnóstico de EPID en promedio fue de 75,4 meses. La duración de la EPID hasta el inicio de tratamiento con CFA fue de 26,8 meses (promedio). Los pacientes 1 y 2 cursaron con fenómenos de Raynaud en 3 fases. Todos los pacientes excepto el 1° presentaron artritis. Tanto tos como dísnea moderada se presentaron inicialmente en el 60 por ciento del total de pacientes. En todos los pacientes, excepto en el 4°, se observó incremento de los reactores de fase aguda. El paciente 1 (mujer) con diagnóstico de ES quien cursó con hipoxemia leve basal normalizó la gasometría a los 6 y 12 meses de tratamiento. Tuvo un recuento citológico del LBA de patrón mixto (neutrófilos, linfocitos y eosinófilos), mejorando el recuento de linfocitos y eosinófilos...