Browsing by Author "Silva, Ana Bento da"
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- An atypical case of focal myositisPublication . Silva, Ana Bento da; Torres, Rita Pinheiro; Lourenço, Maria Helena; Cabral, Catarina; Reis, Rita; Cunha-Branco, Jaime; Gonçalves, Maria JoãoA 64-year-old woman presented with a 10-day history of painful progressive bilateral temporal and right retroauricular region tumefaction, dysphonia, and a body temperature of 37.5°C. She had been previously prescribed antibiotics assuming an oral infection, with no benefit. She had no other complaints and her past medical history and clinical exam were otherwise unremarkable. Her labs revealed high levels of CRP (11.7 mg/dl), ESR (52 mm/h), creatine kinase (CK 623 U/l), myoglobin (83 U/l), aspartate transaminase (56 U/l) and alanine transaminase (69 U/l). Serologies were negative for Treponema pallidum, human immunodeficiency, hepatitis B and C, and Epstein–Barr viruses; blood cultures and immunological study, including anti-nuclear antibodies and antibodies associated with inflammatory myopathies, were negative. A facial MRI revealed thickening and T2/FLAIR hypersignal of the temporal, masseter, and pterygoid muscles with signal intensification after gadolinium injection, suggesting myositis. A temporal muscle biopsy showed marked lymphocyte infiltration (predominantly lymphocyte T CD3), as well as the marking of multiple fibres with major histocompatibility complex class I products, which supported the diagnosis. Prednisolone 0.5 mg/kg/day (30 mg) was started, with marked clinical improvement and normalization of CRP, ESR, and CK.
- Particularly severe form of refractory gastrointestinal involvement in systemic sclerosisPublication . Silva, Ana Bento da; Lourenço, Maria Helena; Cunha-Branco, Jaime; Gonçalves, Maria JoãoA woman with systemic sclerosis presents with a severe and rapidly progressive form of gastrointestinal involvement, mainly marked by recurrent refractory episodes of pseudo-obstruction, culminating in severe malnutrition and dependence on parenteral nutrition. The impact on her quality of life was extremely significant. As a last resort, she started intravenous immunoglobulin with progressive improvement of her symptoms, allowing for the reinstitution of oral diet and removal of parenteral nutrition. After more than 1 year, she maintains clinical stability. Systemic sclerosis has a heterogeneous phenotype, but gastrointestinal involvement is one of the most frequent. Severe manifestations are rare but can lead to severe malnutrition and are associated with high morbidity and mortality rates. Their management is challenging, as the available treatments are still very limited. A better understanding of its pathophysiology, which seems to be unique, is essential to provide more effective treatments and improve quality of life.