Sarcoidosis Presenting as Cranial Multinevritis.

A 29-year-old woman presented to the emergency department with red eyes and hypoacusia since the previous weeks associated with a sudden right facial palsy. Ophthalmologic examination revealed bilateral uveitis and lumbar puncture was compatible with aseptic meningitis. Thoracic computed tomography scan showed pulmonary nodules and mediastinal and hilar lymphadenopathy. Bronchoalveolar lavage revealed a CD4/CD8 ratio of 8, and lymph node biopsy confirmed non-caseating granulomas. The diagnosis of sarcoidosis with ocular, pulmonary, and neurologic involvement was made. Due to sarcoidosis severity at presentation, the patient was started on systemic steroids and methotrexate with improvement. Sarcoidosis involvement of the central nervous system is a rare condition (5-10%) but can be the first manifestation of the disease. Its presentation can be heterogeneous; therefore, symptoms are frequently devalued by patients and physicians, leading to a late diagnosis and permanent damage. Clinicians' awareness is crucial to early diagnosis and treatment.

Implementation of an infection control program with emphasis on cohorting to patients with carbapenemase-producing Enterobacteriaceae. The experience of 2 years in a tertiary teaching hospital in northern Portugal.

BACKGROUND: The emergence of carbapenemase-producing Enterobacterales (CPE) represents a major public health threat. Our purpose was to evaluate a surveillance and cohorting program implemented in patients infected or carriers of CPE. METHODS: A prospective registry of CPE carriers or infected patients was analyzed from October 2015 until December 2017. All inpatients presenting with CPE were included in a hospital cohort with dedicated healthcare staff and contact precaution measures. RESULTS: A total of 480 patients were identified, of which 15.8% (n = 76) were infected. Men comprised 56.7% of the cohort (n = 272) and 69.2% (n = 332) were elderly. About 46.3% (n = 222) had a previous hospital admission and 81.7% (n = 392) had at least 1 antibiotic course in the previous 90 days. There was a decline in infected patients in 2017. Periodic and admission screenings accounted for 63% and 74% of cases in 2016 and 2017, with increased detection rate comparing with contact/investigation screenings. In 2017, significantly fewer patients were identified outside the admission/point of prevalence screening (P = .009). In 2017 the proportion of invasive carbapenem-resistant Klebsiella pneumoniae amongst CPE in our center was below the national average (2016: 13.3% vs 5.2%; 2017: 6.6% vs 8.6%). A reduction of the consumption of carbapenems was also observed in 2017. CONCLUSION: The implementation of the program has increased the number of patients identified by the preventive method and stabilized the emergence of new CPE cases. Furthermore, the program cohort compared well with the national picture, with a lower number of infected patients and a lower proportion of carbapenem-resistant K pneumoniae in invasive specimens. These indicators reflect the added value of the CPE surveillance and cohorting program.

Left Lung and Pulmonary Artery Hypoplasia: A Rare Case of Hemoptysis.

Pulmonary hypoplasia or agenesis is a rare congenital disorder that results in lung underdevelopment. This disease is usually found in children but rarely encountered in adults. We describe the case of an 84-year-old woman diagnosed with a unilateral pulmonary hypoplasia presenting simultaneously with left pulmonary artery hypoplasia. Due to this condition, the patient had a lifelong history of pulmonary infections that resulted in several bronchiectases in the affected lung. Moreover, the pulmonary artery hypoplasia led to the development of pulmonary hypertension and collateral circulation causing hemoptysis, giving rise to the patient attending the emergency department. Although we were able to medically manage the hemoptysis, it can be fatal and require surgical intervention. Hence, an early diagnosis is essential so that appropriate follow-up and prompt prevention and treatment of complications, such as pulmonary infections, hemoptysis and pulmonary hypertension, are achieved. LEARNING POINTS: Although rare and typically associated with childhood, lung and pulmonary artery hypoplasia can in exceptional cases be present in adult life.The diagnosis of lung and pulmonary artery hypoplasia is challenging in adults as they can easily be mistaken for more common diseases.Symptoms and complications may be life-threatening, including dyspnea, hemoptysis, recurrent pulmonary infections and pulmonary hypertension.An early diagnosis is extremely important because it allows a close follow-up with consequent timely detection and treatment of serious complications, especially pulmonary hypertension.

Effectiveness of Tocilizumab in the Treatment of Fasciitis with Eosinophilia: Two Case Reports.

Fasciitis with eosinophilia (FE) is a rare connective tissue disease. Due to its rarity, large-scale studies are lacking, which makes its treatment challenging. Systemic corticosteroids (SCSs) are the cornerstone of treatment; however, additional immunosuppressive drugs (ISDs) are frequently necessary (usually methotrexate). We report 2 patients, for whom an SCS and methotrexate were not a viable long-term option. In the first case, we were unable to taper the SCS dose without symptom relapse, the patient showed only a partial response to methotrexate and presented side effects. The second case never fully responded to the SCS and methotrexate and demonstrated serious SCS adverse effects. Both patients were started on tocilizumab with extremely favourable results, making this drug a potential therapeutic weapon for these patients. LEARNING POINTS: The treatment of FE is challenging and mainly based on retrospective reviews, open-label trials and case reports, all of which included a small number of patients.Currently, systemic corticosteroids are the mainstay of treatment; however, other ISDs are frequently necessary.Cases showing a favourable clinical response to tocilizumab have recently been described in patients with corticosteroid-refractory disease, suggesting that this drug may potentially become a therapeutic weapon for these patients.

Association of severe and therapy-refractory systemic lupus erythematosus and neuromyelitis optica: a management challenge.

Systemic lupus erythematosus (SLE) is a multisystemic autoimmune disorder. Neuromyelitis optica (NMO) is an infrequent neuroinflammatory disorder, whose association with SLE remains rare. The authors report the case of an 18-year-old woman, with SLE refractory to multiple immunosuppressive therapies and novel biological agents. Under immunosuppressive therapy, the patient presented with transverse myelitis with contiguous spinal cord lesions and urinary incontinence, having been diagnosed with seropositive NMO, which was also proven to be refractory to common treatments. Partial recovery of the neurological deficits occurred with plasmapheresis, although not averting the brain involvement by NMO that ensued. The patient was listed nationally for allogeneic bone marrow transplant, but, unfortunately, no match was found and the patient died of severe cerebral NMO flare with coma due to brain swelling and consequent respiratory failure. Although the association of SLE and NMO is very rare, early diagnosis is crucial to facilitate initiation of immunosuppressive therapy.