Cystic fibrosis (CF) is an autosomal recessive genetic disorder characterized by dysfunction of the CFTR gene. It is a multisystem disease that most often affects White individuals. In recent decades, various advances in the diagnosis and treatment of CF have drastically changed the scenario, resulting in a significant increase in survival and quality of life. In Brazil, the current neonatal screening program for CF has broad coverage, and most of the Brazilian states have referral centers for the follow-up of individuals with the disease. Previously, CF was limited to the pediatric age group. However, an increase in the number of adult CF patients has been observed, because of the greater number of individuals being diagnosed with atypical forms (with milder phenotypic expression) and because of the increase in life expectancy provided by the new treatments. However, there is still great heterogeneity among the different regions of Brazil in terms of the access of CF patients to diagnostic and therapeutic methods. The objective of these guidelines was to aggregate the main scientific evidence to guide the management of these patients. A group of 18 CF specialists devised 82 relevant clinical questions, divided into five categories: characteristics of a referral center; diagnosis; treatment of respiratory disease; gastrointestinal and nutritional treatment; and other aspects. Various professionals working in the area of CF in Brazil were invited to answer the questions devised by the coordinators. We used the PubMed database to search the available literature based on keywords, in order to find the best answers to these questions.

A 60-year-old woman received a single 5-mg dose of zoledronic acid (Aclasta) for treatment of postmenopausal osteoporosis. One day after receiving the drug, she acutely developed a painful periorbital swelling, chemosis, and hyperemia in the right eye. The condition worsened despite initial treatment with topical steroids. An orbital CT scan showed right eye proptosis, eyelid edema, and intraorbital fat stranding. A diagnosis of orbital inflammatory disease was made, and the patient was treated with high-dose oral steroids (prednisone 80 mg/day) tapered along 6 weeks. The symptoms and the swelling reduced progressively after initiating oral prednisone, and after 12 days, there was complete resolution of the condition. The patient remained symptom free and had no remission after the treatment interruption.

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