Efficacy of bosentan in patients with refractory thromboangiitis obliterans (Buerger disease): A case series and review of the literature.

The cornerstone of therapy in thromboangiitis obliterans (TAO) is complete abstinence from tobacco. In addition to discontinuation of cigarette smoking, very few pharmacological and surgical options of controversial efficacy are available to date. New therapeutic options with greater efficacy are clearly needed to properly manage these patients.In this preliminary study, we assessed the effectiveness and safety of bosentan in a case series of 8 adults with TAO and severe ischemic ulceronecrotic lesions who were treated with bosentan after inadequate response to platelet inhibitors, vasodilators, and intravenous alprostadil. Additionally, we reviewed 18 well-documented patients with refractory TAO treated with bosentan, which was previously reported (PubMed 1965-2015). These 26 patients formed the basis of our present analysis. All were current smokers.The median duration of bosentan treatment (SD) was 4.5 ±â€Š4 months (range 3-16). Eleven patients (42%) were unable to completely abstain from smoking during their follow-up. With bosentan treatment, no new ischemic lesions were observed in the target extremities. A complete therapeutic response was achieved in 80% of patients, whereas a partial response was observed in 12%. Two patients (8%) ultimately required amputation despite treatment.After discontinuation of bosentan, patients were followed for a median of 20 ±â€Š14 months (range 3-60). Two patients whose trophic lesions had healed relapsed.When comparing patients who gave up smoking with those who were unable to completely abstain from smoking during follow-up, no significant differences were found in efficacy outcomes. Four patients (15%) developed adverse events, requiring bosentan discontinuation in 1 case.These preliminary data suggest that bosentan may be considered a therapeutic option for treatment of cases of severe TAO refractory to conventional treatment, and merit further evaluation in larger controlled, randomized clinical studies.

Nailfold capillaroscopic findings in primary Sjögren's syndrome with and without Raynaud's phenomenon and/or positive anti-SSA/Ro and anti-SSB/La antibodies.

The aim of this study was to assess nailfold capillaroscopic (NC) findings in patients with primary Sjögren's syndrome (PSS) with and without Raynaud's phenomenon (RP) as well as in the presence of positive anti-SSA/Ro and anti-SSB/La antibodies. Videocapillaroscopy was performed in 150 patients with PSS. Data collected included demographics, presence of RP, PSS symptoms, antinuclear antibodies, rheumatoid factor, anti-Ro, anti-La, anti-CCP, salivary scintigraphy, labial biopsy, and NC findings. RP was present in 32% of PSS, keratoconjunctivitis sicca in 91%, oral xerosis in 93%, and skin or genital xerosis in 53%. In patients with positive anti-SSA/Ro (75%) and positive anti-SSB/La (40%), NC showed normal findings in 53% of cases and non-specific in 36%. In patients with PSS, NC was normal in 51% of cases and non-specific in 34%. Scleroderma pattern was found in 14 patients. RP associated with PSS had non-specific capillaroscopy in 40% of cases (p = 0.1). Pericapillary haemorrhages (p = 0.06) and capillary thrombosis (p = 0.2) were not increased, but more dilated capillaries were detected in 48% of cases. Patients with positive anti-Ro and/or anti-La have not a distinct NC profile. Patients with RP associated with PSS had more dilated capillaries, but neither pericapillary haemorrhages nor capillary thrombosis was observed.

Evaluación de una propuesta de criterios de indicación de densitometría ósea en mujeres posmenopáusicas españolas basados en la herramienta FRAX ® / Assessment of a set of FRAX ®-based criteria for the indication of bone densitometry in Spanish postmenopausal women

Fundamento y objetivo: Evaluar el resultado de la aplicación en práctica clínica habitual de unos criterios para la indicación de densitometría ósea en mujeres posmenopáusicas. Pacientes y método: A las pacientes remitidas a una unidad de densitometría ósea desde Atención Primaria que no recibían tratamiento, se les determinó, mediante la herramienta FRAX®, el riesgo de fractura mayor (RFM). Se calculó cuántas exploraciones se hubieran evitado si se hubieran realizado densitometrías únicamente a mujeres ≥65 años con un RFM<10% y mujeres<65 años con un RFM≥3,6%.Resultados: Se incluyeron 643 mujeres con una edad media (DE) de 61 (9) años. El 23% tenía una densidad mineral ósea normal, el 56% tenían osteopenia y el 21%, osteoporosis. El RFM medio fue del 5,9 (5,5)%. En mujeres de edad<65 años, podrían haberse evitado el 64% (273/426) de las densitometrías practicadas. En mujeres≥65 años se podrían haber evitado el 37% (80/217) de las densitometrías realizadas. En total, se podrían haber evitado el 55% (353/643). La sensibilidad del umbral del 3,6% del RFM para el diagnóstico de osteoporosis fue del 51%, la especificidad del 68%, el valor predictivo positivo del 20% y el valor predictivo negativo del 90%. Conclusiones: La aplicación de una propuesta de criterios de indicación de densitometría ósea en mujeres posmenopáusicas basada en la edad y en el riesgo de fractura calculado por FRAX® tendría como consecuencia una disminución significativa de la actividad de la unidad de densitometría ósea (AU)

Background and objective: To assess the effect of the application in routine clinical practice of a proposal of thresholds for the indication of bone densitometry in Spanish postmenopausal women. Patients and methods: We determined the risk of major fracture (RMF) by FRAX® of the patients referred to a bone densitometry unit from Primary Care who were untreated. We calculated how many scans would have been avoided if they had been performed only to women≥65 years with a RMF<10% or women<65 years with a RMF≥3.6%. Results: We included 643 women with a mean age of 61 (9) years. Twenty-three percent had a normal bone mineral density, 56% had osteopenia, and 21% osteoporosis. The RMF was 5.9 (5.5)%. Eighty of 217 (37%) bone scans in women≥65 years and 273 of 426 (64%) in women<65 years would have been avoided. As a whole, 55% of the scans would have been avoided. The sensitivity of the threshold of 3.6% of RMF for the diagnosis of osteoporosis was 51%, specificity 68%, positive predictive value 20%, and negative predictive value 20%. Conclusions: The application of the proposed thresholds for the indication of bone densitometry in Spanish postmenopausal women, based on age and risk of fracture calculated by FRAX® would result in a significant decrease of the activity of the bone densitometry unit (AU)

[Assessment of a set of FRAX(®)-based criteria for the indication of bone densitometry in Spanish postmenopausal women]. / Evaluación de una propuesta de criterios de indicación de densitometría ósea en mujeres posmenopáusicas españolas basados en la herramienta FRAX(®)

BACKGROUND AND OBJECTIVE: To assess the effect of the application in routine clinical practice of a proposal of thresholds for the indication of bone densitometry in Spanish postmenopausal women. PATIENTS AND METHODS: We determined the risk of major fracture (RMF) by FRAX(®) of the patients referred to a bone densitometry unit from Primary Care who were untreated. We calculated how many scans would have been avoided if they had been performed only to women ≥ 65 years with a RMF < 10% or women<65 years with a RMF ≥ 3.6%. RESULTS: We included 643 women with a mean age of 61 (9) years. Twenty-three percent had a normal bone mineral density, 56% had osteopenia, and 21% osteoporosis. The RMF was 5.9 (5.5)%. Eighty of 217 (37%) bone scans in women ≥ 65 years and 273 of 426 (64%) in women<65 years would have been avoided. As a whole, 55% of the scans would have been avoided. The sensitivity of the threshold of 3.6% of RMF for the diagnosis of osteoporosis was 51%, specificity 68%, positive predictive value 20%, and negative predictive value 20%. CONCLUSIONS: The application of the proposed thresholds for the indication of bone densitometry in Spanish postmenopausal women, based on age and risk of fracture calculated by FRAX(®) would result in a significant decrease of the activity of the bone densitometry unit.

The activity of a Spanish bone densitometry unit revisited under the point of view of FRAX

Objetivo. Analizar el riesgo de fractura calculado por FRAX y sus determinantes en los pacientes remitidos a una unidad de densitometría ósea para evaluación de la densidad mineral ósea (DMO). Métodos. Los pacientes remitidos desde Atención Primaria a la Unidad de Densitometría para evaluación de la DMO rellenaron un cuestionario autoadministrado acerca de los factores de riesgo clínicos incluidos en el FRAX; se les realizó una densitometría ósea. Con los datos del cuestionario, se analizó el riesgo absoluto de presentar una fractura mayor (MFR) y de cadera (HFR). Ambos riesgos se calcularon con o sin la inclusión de la DMO en el algoritmo: MFR+, MFR-, HFR+ y HFR-. Resultados. Se analizaron los datos de 853 mujeres con una edad media de 61,9 (8,9) años y un índice de masa corporal medio de 27,0 (4,2) kg/m2. El 20% de las pacientes tenía una DMO normal, el 55% tenía osteopenia y el 25%, osteoporosis. Excluyendo la edad y el índice de masa corporal, el número de factores de riesgo de fractura fue bajo. El MFR+ medio fue de 5,4 (4,8)%; el MFR- de 6,3 (5,5)%; el HFR+, de 1,5 (2,9)%; y el HFR- de 2,1 (3,3)%. Cuando se incluyeron los valores densitométricos en el algoritmo de cálculo del riesgo de fractura, éste fue significativamente menor (p < 0,001), especialmente en pacientes con mejor DMO. Conclusiones. En nuestro medio, el riesgo de fractura calculado por FRAX en las pacientes remitidas a la unidad de densitometría para evaluación de la DMO es bajo. El riesgo de fractura es inferior cuando se introduce la DMO en el algoritmo de cálculo (AU)

In March 2008, FRAX, developed by Kanis and collaborators in the University of Sheffield and supported by the World Health Organization, became available online to calculate absolute risk of osteoporotic fracture in the next 10 years. Objective. To analyze the risk of fracture calculated by FRAX and its determinants in the patients sent to a densitometry unit for bone mineral density (BMD) testing. Methods. All the patients submitted by Primary Care to the Densitometry Unit for BMD testing underwent a self administered questionnaire to assess the clinical risk factors included in FRAX and a bone densitometry of lumbar spine and proximal femur with a DXA densitometer Hologic QDR 4500. They were classified as having a normal BMD, osteopenia or osteoporosis along with the recommendations of the International Society for Clinical Densitometry. As the reference population to calculate the T and Z scores, we used the one from the NHANES III study for femoral neck and total hip and the one from the Study of the Spanish Population for total spine. With the data of the questionnaire, we calculated, by FRAX, the absolute risk in the next ten years of having a major fracture (MFR) or a hip fracture (HFR). Both risks were calculated with or without the inclusion in the algorithm of BMD: MFR+, MFR−, HFR+ and HFR−. The results were recorded in an Access 2003 database and analyzed with the statistical package SPSS 15.0 for Windows. Results. We analyzed the data from 853 women with a mean age of 61.9 (8.9) years and a mean body mass index of 27.0 (4.2)kg/m2. Mean BMD at lumbar spine was 0.873 (0.127)g/cm2; at femoral neck, 0.704 (0.105)g/cm2; and at total hip, 0.817 (0.107)g/cm2. Twenty percent of the patients had a normal BMD, 55% had osteopenia and 25%, osteoporosis. Yet excluding age and body mass index, the number of fracture risk factors seems low: 31% of the patients had no risk of fracture; 40%, had one; 22%, two; 6%, three; 1%, four; and one patient had five. Mean MFR+ was 5.4 (4.8)%; mean MFR−, 6.3 (5.5)%; mean HFR+, 1.5 (2.9)%; and HFR−, 2.1 (3.3)%. When BMD was included in the algorithm for the calculation of the risk of fracture, the risk was statistically lower (p<0.001), especially in patients with better BMD. Conclusions. The risk of fracture calculated by FRAX in the patients sent to a densitometry unit for bone BMD testing seems low and, probably, a better selection of the patients would detect a higher risk of fracture population. When the fracture risk is calculated with the introduction of BMD in the algorithm, it is lower than without including BMD (AU)

The activity of a Spanish bone densitometry unit revisited under the point of view of FRAX.

UNLABELLED: In March 2008, FRAX, developed by Kanis and collaborators in the University of Sheffield and supported by the World Health Organization, became available online to calculate absolute risk of osteoporotic fracture in the next 10 years. OBJECTIVE: To analyze the risk of fracture calculated by FRAX and its determinants in the patients sent to a densitometry unit for bone mineral density (BMD) testing. METHODS: All the patients submitted by Primary Care to the Densitometry Unit for BMD testing underwent a self administered questionnaire to assess the clinical risk factors included in FRAX and a bone densitometry of lumbar spine and proximal femur with a DXA densitometer Hologic QDR 4500. They were classified as having a normal BMD, osteopenia or osteoporosis along with the recommendations of the International Society for Clinical Densitometry. As the reference population to calculate the T and Z scores, we used the one from the NHANES III study for femoral neck and total hip and the one from the Study of the Spanish Population for total spine. With the data of the questionnaire, we calculated, by FRAX, the absolute risk in the next ten years of having a major fracture (MFR) or a hip fracture (HFR). Both risks were calculated with or without the inclusion in the algorithm of BMD: MFR+, MFR-, HFR+ and HFR-. The results were recorded in an Access 2003 database and analyzed with the statistical package SPSS 15.0 for Windows. RESULTS: We analyzed the data from 853 women with a mean age of 61.9 (8.9) years and a mean body mass index of 27.0 (4.2)kg/m(2). Mean BMD at lumbar spine was 0.873 (0.127)g/cm(2); at femoral neck, 0.704 (0.105)g/cm(2); and at total hip, 0.817 (0.107)g/cm(2). Twenty percent of the patients had a normal BMD, 55% had osteopenia and 25%, osteoporosis. Yet excluding age and body mass index, the number of fracture risk factors seems low: 31% of the patients had no risk of fracture; 40%, had one; 22%, two; 6%, three; 1%, four; and one patient had five. Mean MFR+ was 5.4 (4.8)%; mean MFR-, 6.3 (5.5)%; mean HFR+, 1.5 (2.9)%; and HFR-, 2.1 (3.3)%. When BMD was included in the algorithm for the calculation of the risk of fracture, the risk was statistically lower (p<0.001), especially in patients with better BMD. CONCLUSIONS: The risk of fracture calculated by FRAX in the patients sent to a densitometry unit for bone BMD testing seems low and, probably, a better selection of the patients would detect a higher risk of fracture population. When the fracture risk is calculated with the introduction of BMD in the algorithm, it is lower than without including BMD.

Pancreatitis, panniculitis, and polyarthritis.

BACKGROUND AND OBJECTIVE: Lobular panniculitis, together with polyarthritis and intraosseous fat necrosis, may occasionally complicate pancreatic disease. This triad is known in the literature as the pancreatitis, panniculitis, and polyarthritis (PPP syndrome). We describe a case of the PPP syndrome and review the available literature to summarize the clinical characteristics of patients with this condition. METHODS: A patient with the PPP syndrome, with evidence of extensive intraosseous fat necrosis in the joints involved revealed by magnetic resonance imaging, is described and the relevant literature based on a PubMed search from 1970 to February 2008 is reviewed. The keywords used were pancreatitis or pancreatic disease, panniculitis, arthritis, and intraosseous fat necrosis. RESULTS: Including our case, 25 well-documented patients with the PPP syndrome have been reported. Our patient had few abdominal symptoms despite high serum levels of pancreatic enzymes. In our review of the literature, almost 2/3 of patients had absent or mild abdominal symptoms, leading to misdiagnosis. The delay in diagnosis and specific treatment of the underlying pancreatitis worsens the prognosis of this condition, which has a mortality rate as high as 24%. In nearly 45% of the patients, the arthritis follows a chronic course with a poor response to nonsteroidal anti-inflammatory drugs and corticosteroids, and the rapid development of radiographic joint damage. CONCLUSION: Certain forms of pancreatic disease can very occasionally cause arthritis and panniculitis. Although uncommon, physicians should be alert to the possible presence of this syndrome for 2 reasons: first, unrecognized pancreatic disease can be fatal if not treated promptly; second, to avoid inappropriate and risky therapy to improve joint symptoms.

Multicentric Castleman's disease mimicking adult-onset Still's disease.

Castleman's disease is a rare lymphoproliferative disorder having two types of presentation: the localized and the multicentric form. Multicentric Castleman's disease (MCD) typically presents with constitutional symptoms, generalized peripheral lymphadenopathy, hepatosplenomegaly, and laboratory markers of inflammation. Rash and arthritis may also be initial complaints of this disease. In these cases, MCD can resemble adult-onset Still's disease (AOSD), especially if the arthritis precedes other manifestations. We describe a patient with initial clinical suspicion of AOSD. Eighteen months later evidence of MCD was ascertained when the patient developed insidiously growing axillary lymphadenopathies. Despite its rarity, MCD should be borne in mind in the differential diagnosis of patients with suspicion of AOSD.