Autor(es): Mendonça, P ; Taulaigo, AV ; Caetano, A ; Moraes-Fontes, MF
Data: 2019
Identificador Persistente: http://hdl.handle.net/10400.17/3463
Origem: Repositório do Centro Hospitalar de Lisboa Central, EPE
Assunto(s): Antibodies, Monoclonal, Humanized; Bone Resorption; Calcium; Diagnosis, Differential; Dietary Supplements; Female; Humans; Malnutrition; Mandible; Middle Aged; Scleroderma, Systemic; Tomography, X-Ray Computed; Tracheostomy; Vitamin D; Vocal Cord Paralysis; HCC DAUTOIM; HCC IMA
Sudden respiratory distress in association with severe weight loss are unusual features of systemic sclerosis (SSc). We report the case of a 56-year-old Caucasian woman with a 9-year history of a diffuse form of SSc who presented with acute stridor due to vocal cord paralysis and required an emergency tracheostomy. She had sought medical attention only after 4 years of disease onset, presenting with a mask-like face, diffuse skin thickening, acro-osteolysis and severe interstitial lung disease. Even though skin tightness improved after immunosuppressive treatment, several spontaneous facial fractures and episodes of dysphagia and choking occurred in the years that followed. At the time of stridor, she was severely malnourished and a percutaneous endoscopic gastrostomy was required for feeding. Permanent vocal cord damage in combination with severe loco-regional bone resorption resulted in severe disability and impaired nutrition. We hereby highlight the features of SSc for which therapy remains challenging.
