P.056 Optimizing IVIg Use for Neuromuscular Conditions in British Columbia, Canada – Targeting High and Chronic User Groups

Background: Neuromuscular conditions account for 1/3 of IVIg use in BC and costs over $10 million annually. Since 2013, the BC Neuromuscular Review Panel has developed diagnostic and treatment algorithms for the use of IVIg. A framework was created to review high dose and chronic users. Methods: Utilizing Central Transfusion Registry data, all patients treated with IVIg for approved neuromuscular conditions (CIDP, MG, MMN) since April 1, 2013 were identified. Annual cohorts for patients using higher than usual dose and chronic use (>3 years) were established, and evaluated annually. Patient specific recommendations were made. Results: The initial cohort identified 38 high users of 377 patients receiving IVIg. 27 appropriate, 9 “not appropriate”. Subsequent cohorts showed a decrease in number of patients receiving inappropriate IVIg doses. In BC there has been a 36% increase in neuromuscular patients treated with IVIg (377 in 2013/14 to 512 in 2016/17). Despite this, IVIg the program has effectively reduced the annual grams/patient from 516 gm/patient in 2013/14 to 489 gm/patient in 2016/17. Conclusions: The BC Neuromuscular IVIg Review confirms that the majority of IVIg use is appropriate. Following yearly cohorts of chronic and high dose users helps optimize IVIg use, which may lead to improved patient care.

Caso 2/19: “Uma doença destruidora da face” / Case 2/19: “A face-destroying disease”

Caso relatado na Reunião de Discussão de Casos Clínicos do Hospital Universitário Prof. Polydoro Ernani de São Thiago, iniciada pelos Profs. Jorge Dias de Matos, Marisa Helena César Coral e Rosemeri Maurici da Silva, em julho de 2017. No dia 13 de junho de 2019, no bloco do curso de medicina, realizou-se a apresentação e discussão do caso cujo registro é apresentado a seguir: um paciente de 38 anos é acompanhado no ambulatório de reumatologia, com uma doença destrutiva das vias aéreas superiores, pansinusopatia, ANCA e anti-proteinase 3 positivos. É usuário crônico de cocaína inalável e de corticosteróides. Um dia, dá entrada na emergência com cefaleia aguda e meningismo. Qual é o diagnóstico?Case reported at the Clinical Cases Discussion Meeting of the University Hospital “Polydoro Ernani of São Thiago”, initiated by Profs. Jorge Dias de Matos, Marisa Helena César Coral and Rosemeri Maurici da Silva, July 2017. On June 13, 2019, in the medical school block, the case was presented and discussed: A 38-year-old male patient is followed at the rheumatology outpatient clinic with a destructive upper airway disease, pansinusopathy, positive ANCA and anti-proteinase 3. He is a chronic user of inhaled cocaine and corticosteroids. One day, he comes to the emergency room with acute headache and meningism. What is the diagnosis?

Pulmonary foreign body granulomatosis in a chronic user of powder cocaine

ABSTRACT We describe the case of a 33-year-old man, a chronic user of powder cocaine, who presented with dyspnea, fever, night sweats, and significant weight loss. Chest HRCT revealed centrilobular nodules, giving an initial impression of miliary tuberculosis. Therefore, he was started on an empirical, four-drug antituberculosis treatment regimen. Four weeks later, despite the tuberculosis treatment, he continued to have the same symptoms. We then performed transbronchial lung biopsy. Histopathological analysis of the biopsy sample revealed birefringent foreign body granuloma. A corroborative history of cocaine snorting, the presence of centrilobular nodules, and the foreign body-related histopathological findings led to a diagnosis of pulmonary foreign body granulomatosis. This report underscores the fact that pulmonary foreign body granulomatosis should be included in the differential diagnosis of clinical profiles resembling tuberculosis.

Disseminated cryptococcosis presenting as cutaneous cellulitis in an adolescent with systemic lupus erythematosus

We report here the case of a 17-year-old girl from Pelotas, Brazil, with systemic lupus erythematosus and disseminated cryptococcal infection. Prior to diagnosis, she was a chronic user of corticosteroids and other immunosuppressive drugs. Her first symptoms were skin lesions that simulated bacterial cellulitis. Upon suspicion, we performed a biopsy and fungal infection was confirmed. Appropriate therapy was established, and the patient was discharged after 42 days of treatment in complete remission.