Scleredema diabeticorum in a patient: un uncommon etiology of restrictive lung pattern Escleredema diabeticorum en un paciente: una etiología infrecuente de patrón restrictivo pulmonar.

Scleredema adultorum of Buschke is a rare skin disease characterized by skin thickening and tightening typically at the neck and the upper part of the body. This thickening results from increased mucin deposition in the reticular dermis. Three variants are recognized. Scleredema diabeticorum is one subtype associated with diabetes mellitus. We report a man with a history of poorly controlled diabetes presenting with extensive scleredema adultorum of the trunk in a "cuirasse" pattern associated with restrictive lung disease. Cutaneous ultrasonography revealed a marked thickening of the dermis as well as various echogenic spots in the dermis. Cutaneous hardness secondary to scleredema may cause limited mobility. Therefore, respiratory manifestations may be associated and assessed, mainly in cases of extensive scleredema involving trunk and shoulders. Moreover, cutaneous ultrasonography may be useful to monitor the evolution of scleredema and confirm the diagnosis.

Neumonitis por citomegalovirus durante el tratamiento crónico con metotrexato / Pneumonitis due to cytomegalovirus during chronic methotrexate treatment

Si bien la neumonitis por hipersensibilidad es la complicación pulmonar más frecuentemente descrita durante el tratamiento con metotrexato, existen otras complicaciones de tipo linfoproliferativo e infeccioso que hay que tener en cuenta dentro del diagnóstico diferencial ante una neumopatía en el contexto de dicho tratamiento. La existencia de un riesgo incrementado de desarrollar procesos infecciosos o reactivaciones de microorganismos latentes puede ser similar al observado a lo largo del tratamiento con antagonistas del factor de necrosis tumoral y corticoides. Dentro de ellas, la neumonía por citomegalovirus es una complicación muy severa a tener en cuenta, ya que el diagnóstico acertado y el tratamiento oportuno evitarán un desenlace potencialmente mortal (AU)

Although hypersensitivity pneumonitis is the most common pulmonary complication described during treatment with methotrexate, other complications like lymphoproliferative and infectious disease may be considered in the study of respiratory disease associated to methotrexate. The existence of an increased risk to developing infectious diseases may be similar to that observed during treatment with antagonists of tumor necrosis factor and corticosteroids, where Cytomegalovirus pneumonia is a serious complication; early diagnosis and treatment will prevent a potentially fatal outcome (AU)

Pneumonitis due to cytomegalovirus during chronic methotrexate treatment.

Although hypersensitivity pneumonitis is the most common pulmonary complication described during treatment with methotrexate, other complications like lymphoproliferative and infectious disease may be considered in the study of respiratory disease associated to methotrexate. The existence of an increased risk to developing infectious diseases may be similar to that observed during treatment with antagonists of tumor necrosis factor and corticosteroids, where Cytomegalovirus pneumonia is a serious complication; early diagnosis and treatment will prevent a potentially fatal outcome.

Reducción de tamaño del angiomiolipoma renal tras el tratamiento con everolimus en trasplante pulmonar por linfangioleiomiomatosis / Reduction in Size of Renal Angiomyolipoma after Treatment with Everolimus in Lung Transplantation due to Lymphangioleiomyomatosis

La linfangioleiomiomatosis (LAM) es una enfermedad rara caracterizada por la proliferación anormal de células musculares lisas inmaduras y una destrucción quística del pulmón, que condiciona el pronóstico de la enfermedad. Los angiomiolipomas renales suelen ser muy frecuentes en esta enfermedad, generalmente de curso asintomático, salvo complicaciones. Ante la ausencia de un tratamiento curativo, las últimas publicaciones reflejan resultados esperanzadores en la terapia molecular para evitar el deterioro funcional y el control del tamaño de los angiomiolipomas. Entre estas terapias destacan los inhibidores del complejo mTOR, sobre todo sirolimus. Presentamos un caso clínico de una paciente diagnosticada de LAM sometida a trasplante pulmonar con reducción del tamaño del angiomiolipoma renal tras el tratamiento con el inhibidor mTOR everolimus(AU)

Lymphangioleiomyomatosis (LAM) is a rare disease characterized by abnormal proliferation of immature smooth muscle cells and cystic lung destruction, which determines the prognosis of the disease. The kidney angiomyolipomas are usually very common in this disease and are usually asymptomatic unless complications arise. In the absence of a curative treatment, recent publications show promising results in molecular therapy to prevent functional decline and to control the size of the angiomyolipomas. These therapies include mTOR complex inhibitors, especially sirolimus. We report a case of a patient diagnosed with LAM who underwent lung transplantation with reduction of renal angiomyolipoma size after treatment with the mTOR inhibitor everolimus(AU)

Reduction in size of renal angiomyolipoma after treatment with everolimus in lung transplantation due to lymphangioleiomyomatosis.

Lymphangioleiomyomatosis (LAM) is a rare disease characterized by abnormal proliferation of immature smooth muscle cells and cystic lung destruction, which determines the prognosis of the disease. The kidney angiomyolipomas are usually very common in this disease and are usually asymptomatic unless complications arise. In the absence of a curative treatment, recent publications show promising results in molecular therapy to prevent functional decline and to control the size of the angiomyolipomas. These therapies include mTOR complex inhibitors, especially sirolimus. We report a case of a patient diagnosed with LAM who underwent lung transplantation with reduction of renal angiomyolipoma size after treatment with the mTOR inhibitor everolimus.

Association of cellular adhesion molecules and oxidative stress with endothelial function in obstructive sleep apnea.

OBJECTIVE: To evaluate the impact of oxidative stress and cellular adhesion molecules on ischemic reactive hyperemia (IRH) in patients with OSA. MATERIALS AND METHODS: Consecutive patients treated at a sleep laboratory and whose polysomnography showed an apnea hypopnea index (AHI) ≥5 were included in the study. Patients with acute illness receiving vasoactive medications were excluded. Based on their oxygen desaturation index (ODI), subjects were assigned to the mild-moderate (ODI ≤30) or the severe desaturation group (ODI >30). Then IRH and oxidative stress markers [malondialdehyde (MDA)] and proinflammatory markers (ICAM-1 and P-selectin) were measured. RESULTS: Sixty-eight subjects with OSA were included, 31 in the mild-moderate desaturation group and 37 in the severe group. No differences by age, gender and body mass index were observed. The severe desaturation group showed significantly higher values in the AHI, MDA, ICAM-1 and P-selectin (p<0.005), as well as a worsening of IRH (p=0.001). Only ICAM-1 (p=0.019) and P-selectin (p=0.033) were independently associated with IRH in a multiple-linear regression model. CONCLUSION: Patients with OSA and greater intermittent hypoxia showed worse endothelial function, and higher levels of MDA, ICAM-1 and P-selectin. Nevertheless, ICAM-1 and P-selectin rather than MDA were independently associated with IRH.