RESUMEN
A 37-year-old woman with rheumatoid arthritis and interstitial lung disease (ILD) developed clinically amyopathic dermatomyositis (CADM) after achieving pregnancy through in vitro fertilization. She was given oral prednisolone, which improved her respiratory status, and delivered a healthy baby at 35 weeks' gestation. There are few reports of successful outcomes for CADM during pregnancy; to the best of our knowledge, this is the first report of successful delivery in a patient with both CADM and ILD.
Asunto(s)
Dermatomiositis/diagnóstico , Enfermedades Pulmonares Intersticiales/diagnóstico , Complicaciones del Embarazo/diagnóstico , Adulto , Dermatomiositis/complicaciones , Femenino , Humanos , Nacimiento Vivo , Enfermedades Pulmonares Intersticiales/complicaciones , EmbarazoRESUMEN
The diagnosis of macrophage activation syndrome (MAS) in patients with systemic lupus erythematosus (SLE) may be challenging as it can mimic the clinical features of the underlying disease or be confused with an infectious complication. In this report, a Japanese woman in her forties had diverse clinical features of MAS at initial presentation of SLE, where erythematosus plaques with histiocytic infiltrates focally surrounding degenerated collagen might be the earliest indicator of MAS.
Asunto(s)
Lupus Eritematoso Sistémico/complicaciones , Síndrome de Activación Macrofágica/patología , Macrófagos/patología , Adulto , Femenino , Humanos , Lupus Eritematoso Sistémico/patología , Síndrome de Activación Macrofágica/complicacionesRESUMEN
Systemic sclerosis (SSc) is a multi-organ fibrotic disease that affects the skin and various internal organs. Therapeutic strategies for tissue fibrosis have not been established; however, aberrantly activated fibroblasts in affected lesions are key targets for modulating fibrosis. Recently, increased intracellular cyclic GMP (cGMP) levels were demonstrated to improve fibrosis levels in various diseases. The purpose of this study was to assess the anti-fibrotic properties of cGMP in cultured fibroblasts from patients with SSc. The phosphodiesterase (PDE) 5 inhibitor sildenafil increased the intracellular cGMP levels in skin fibroblasts in a dose-dependent manner. Sildenafil treatment also significantly decreased the expression of several pro-fibrotic factors that were upregulated by TGF-ß1 treatment in SSc skin fibroblasts. These inhibitory effects occurred via non-canonical TGF-ß signaling. Our findings revealed that sildenafil might be a novel strategy to treat tissue fibrosis and vasculopathy in SSc.