Mariana Brandão

Systemic Lupus Erythematosus, Progressive Multifocal Leukoencephalopathy, and T-CD4+ Lymphopenia

Progressive multifocal leukoencephalopathy (PML) is a rare opportunistic infection caused by the reactivation of JC virus and occurs in patients with severe primary or secondary immunosuppression. Recently, PML is becoming relevant in autoimmune disorders, particularly in patients treated with biologic agents. However, systemic lupus erythematosus (SLE) appears to be associated with susceptibility to PML that cannot be entirely explained by the immunosuppressive therapy. The authors present two patients with the diagnosis of SLE and PML: One had a heavy immunosuppressive therapy history, and the other had never experienced biologic or cytotoxic therapeutics. Both patients had a profound T-CD4+ lymphopenia during their clinical history. These two cases emphasize the importance of CD4+ lymphopenia in SLE patients with and without immunosuppressors regarding opportunistic infections.

Idiopathic inflammatory myopathies: definition and management of refractory disease.

Adult idiopathic inflammatory myopathies, commonly referred to as myositis, are a heterogeneous group of diseases with an autoimmune etiology. In this review, the authors are going to focus on myositis excluding inclusion body myositis. They will review the prognostic factors (for mortality and response to steroids), define refractory disease, introduce a new concept (presumed refractory disease), analyze definitions of active disease, damage and improvement criteria, and summarize therapeutic alternatives for refractory patients, based on different disease phenotypes.

Hypertrophic Cranial Pachymeningitis and Skull Base Osteomyelitis by Pseudomonas Aeruginosa: Case Report and Review of the Literature

Hypertrophic cranial pachymeningitis (HCP) is an uncommon disorder characterized by localized or diffuse thickening of the dura mater, and it usually presents with multiple cranial neurophaties. It has been associated with a variety of inflammatory, infectious, traumatic, toxic and neoplasic diseases, when no specific cause is found the process is called idiopathic. The infectious cases occur in patients under systemic immunosuppression, which have an evident contiguous source or those who have undergone neurosurgical procedures. We describe a case of a 62-year-old immunosuppressed woman with diabetes and rheumatoid arthritis, which had HCP and osteomyelitis of the skull base caused by pseudomonas aeruginosa, presenting with headache and diplopia. We believe this is the second documented case of pachymeningitis secondary to this microorganism. As a multifactorial disease, it is essencial to determine the specific causative agent of HCP before making treatment decisions, and great care is needed with immunocompromised patients. Keywords Pseudomonas aeruginosa; Hypertrophic pachymeningitis; Ophtalmoplegia, optical neuropathy; Osteomyelitis; Skull base

The Challenge of Tuberculosis Infection Related to Adalimumab: A Subtle and Rare Presentation

With the increasing use of biological therapies, there was a concomitant increase in concern and sensitivity for associated opportunistic infections. There has been growing evidence that anti-tumor necrosis factor (TNF) therapy increases the risk of opportunistic infection. Screening for latent tuberculosis (TB) prior to initiation of therapy has reduced the incidence of active TB, although it is not completely preventable. The possibility of active TB should always be considered in immunosuppressed patients under anti-TNF therapy. We report a case of muscular TB with psoas abscess in a patient under adalimumab. This case highlights the importance of the high suspicion threshold for Mycobacterium tuberculosis infection, in patients with subtle symptoms. J Med Cases. 2018;9(1):23-25 doi: https://doi.org/10.14740/jmc2979w

Cerebral Nocardiosis in a Patient With Rheumatoid Arthritis Under Treatment With Tocilizumab

The use of biological therapy has revolutionized the treatment of various autoimmune diseases. Tocilizumab (TCZ) is a recombinant humanized antiinterleukin-6 (IL-6) receptor monoclonal antibody approved for the treatment of patients with rheumatoid arthritis, as monotherapy or in combination with other disease-modifying anti-rheumatic drugs (DMARDs). Clinical and post-marketing surveillance studies of TCZ indicate that infections are the most frequent adverse events. Most of the serious infections described are bacterial and viral, with no uncommon pathogens. The authors report a case of a patient with destructive rheumatoid arthritis under TCZ that presented with neurological symptoms and a right cerebellar lesion with marked diffusion restriction suggestive of cerebellar abscess. The microbiologic studies from aspirative cerebral biopsy revealed Nocardia farcinica . With adequate antibiotics, the patient evolved with resolution of all symptoms and brain abscess at 5 months of follow-up. According to published reports, this is the first case of nocardial infection in patients under TCZ. The case highlights the importance of being aware of risk infection in these patients and need of an early diagnosis allowing a prompt treatment. J Med Cases. 2018;9(2):68-72 doi: https://doi.org/10.14740/jmc2995w

Tuberculous osteomyelitis of the wrist presenting as a monoarthritis syndrome

Osteoarticular tuberculosis is the third most frequent location of tuberculosis after the lung and lymph nodes, accounting for approximately 10%–20% of all cases of extrapulmonary disease. Tuberculosis of the hand and wrist is the rarest osteoarticular location after the shoulder. The authors report the case of a 50-year-old woman without medical history who was diagnosed with isolated tuberculosis of the wrist presenting as monarthritis. The diagnosis was confirmed by histopathological and microbiological examination. Late stages of osteomyelitis are even rarer without any predisposing factors such as immunosuppression. This case underlines the importance of including tuberculosis in the differential diagnosis of monoarthritic syndromes to prevent delayed initiation of therapy and consequent complications and bone damage.

The National Student Survey: validation in Portuguese medical students

Abstract The UK National Student Survey (NSS) is a sound and widely used instrument for assessing students’ academic experiences. We aimed to translate the NSS for Portuguese students and to validate the instrument in a sample of medical undergraduates. The research team translated and adapted the NSS for Portuguese students (NSS-P). The survey was administered on an online platform to 1,256 final-year students at eight Portuguese medical schools. A total of 329 medical students (69.9% female) replied to the NSS-P, a response rate of 26.2%. Confirmatory factor analysis showed that the original six-factor structure had an adequate fit to the data. Adequate internal consistency was observed for all the subscales. Medium to large correlations were found among all the subscale scores and between the subscale scores and the students’ overall satisfaction. Multiple regression showed that the scores on the Teaching, Organization and Management and Personal Development subscales significantly predicted the students’ overall satisfaction. Approximately 64% of the students reported being satisfied with the quality of their courses. Significant differences among the medical schools in their NSS-P scores were found. The NSS-P is a valid and reliable measure for assessing medical students’ perceptions of academic quality.

A Rare Presentation of Leptospirosis

Leptospirosis has a wide spectrum of clinical manifestations. Acute renal failure, an important complication, generally involves interstitial and tubular damage. We describe the case of a 42-year-old man who was admitted with fever, back pain and periorbital oedema. He had hypertension, thrombocytopenia, acute renal failure, hypoalbuminaemia, hypertriglyceridaemia and proteinuria >4.00 g/l. The renal biopsy showed mesangioproliferative glomerulonephritis. Due to the epidemiological context and clinical picture, ceftriaxone was started with rapid clinical improvement. Blood PCR for leptospira came back positive. The presentation of leptospirosis as nephrotic syndrome is rare and this diagnosis should be considered before performing a renal biopsy. LEARNING POINTS Leptospirosis is a zoonosis with a wide spectrum of clinical manifestations including acute renal failure, which is an important complication. Leptospirosis should be considered in a patient with nephrotic syndrome and acute renal failure if the epidemiological context is favourable. Leptospirosis is one of the causes of nephrotic syndrome where a renal biopsy is not indicated.

Satisfação com o Internato Médico em Portugal

INTRODUCTION In the last years, the global context of medical education and Medical Residency programs in Portugal suffered substantial changes. The primary objective of this study was to evaluate and characterize medical residents ́ satisfaction with medical residency programs in Portugal and to identify features that could be improved. MATERIAL AND METHODS We utilized as model the survey Postgraduate Hospital Educational Environment Measure that has been developed in the United Kingdom and is speci cally targeted to medical residents. The survey was translated and adapted to the Portuguese reality. The survey was available online during April and May of 2016. RESULTS A total of 3456 responses were obtained, corresponding to a response rate of 35%. Endocrinology/Nutrition, Cardiology, Anesthesiology, Family Physician and Gastroenterology were the specialties in which the degree of satisfaction was higher, while Forensic Medicine, Medical Oncology, Internal Medicine, General Surgery and Pneumology showed the lowest level of satisfaction. DISCUSSION This study presented a high response rate when compared to previous studies. Portuguese medical residents presented high levels of satisfaction. Depending on year of medical residency, region, type of specialty and type of hospital marked asymmetries were noticed. CONCLUSION The survey ́s results should constitute in the future a support tool for the implementation of local and national measures relating to the medical residency. It is advisable to regularly conduct satisfaction surveys to medical residents.