Pulmonary mucormycosis following autologous hematopoietic stem cell transplantation for rapidly progressive diffuse cutaneous systemic sclerosis: A case report.

RATIONALE: The use of autologous hematopoietic stem cell transplantation (AHSCT) for autoimmune diseases has become the first indication for transplant in nonmalignant disease. Mucormycosis is a rare invasive infection with increasing incidence in patients treated with AHSCT. We report the first case of pulmonary mucormycosis following AHSCT for systemic sclerosis (SSc). PATIENT CONCERNS: A 24-year-old woman with rapidly progressive diffuse cutaneous SSc presented with an acute respiratory distress syndrome 6 days after AHSCT. DIAGNOSES: The results of clinical and computed tomography scan were consistent with pulmonary mucormycosis and the diagnosis was confirmed by a positive Mucorales Polymerase Chain Reaction on a peripheral blood sample. INTERVENTIONS AND OUTCOMES: Early antifungal therapy by intravenous amphotericin B provided rapid improvement within 4 days and sustained recovery after 2 years of follow-up. LESSONS: With the progressively increasing use of AHSCT and other stem cell therapy for treatment of severe SSc and other autoimmune diseases, the potential onset of rare post-transplant fungal infections, such as mucormycosis, requires careful patient monitoring and better awareness of early initiation of adequate therapy.

Improving communication between the general practitioner and the oncologist: a key role in coordinating care for patients suffering from cancer.

BACKGROUND: Patients suffering from cancers are increasingly numerous in general practice consultations. The General Practitioner (GP) should be at the heart of the management of patients. Several studies have examined the perceptions of GPs confronted with the patient suffering from cancer and the relationships of GPs with oncologists, but few studies have focused on the patients' perspective. We studied the three-way relationship between the oncologist, the GP, and the patient, from the patient's point of view. METHODS: A questionnaire validated by a group consisting of GPs, oncologists, nurses, an epidemiologist and quality analyst, was administered over a three-week period to patients suffering from cancer receiving chemotherapy in a day hospital. RESULTS: The analysis was based on 403 questionnaires. Patients had confidence in the GP's knowledge of oncology in 88% of cases; 49% consulted their GP for pain, 15% for cancer-related advice, and 44% in emergencies. Perceived good GP/oncologist communication led patients to turn increasingly to their GP for cancer-related consultations (RR = 1.14; p = 0.01) and gave patients confidence in the GP's ability to manage cancer-related problems (RR = 1.30; p < 0.01). Mention by the oncologist of the GP's role increased the consultations for complications (RR = 1.82; p < 0.01) as well as recourse to the GP in an emergency (RR = 1.35; p < 0.01). CONCLUSION: Patients suffering from cancer considered that the GP was competent, but did not often consult their GP for cancer-related problems. There is a discrepancy between patients' beliefs and their behaviour. When the oncologist spoke to patients of the GP's role, patients had recourse to their GP more often. Systematically integrating a GP consultation to conclude cancer diagnosis disclosure, could improve management and care coordination.

High rate of indeterminate results of the QuantiFERON-TB Gold in-tube test, third generation, in patients with systemic vasculitis.

OBJECTIVES: To describe the frequency of QuantiFERON-TB Gold in-tube test® (QFT-GIT) indeterminate results due to no response to phytohaemagglutinin A stimulation in the control tube in vasculitis patients prior to immunosuppressant therapy; and to compare it with other groups of patients. METHODS: This was a single-centre, retrospective study. Patients and controls were included between 1 January 2008 and 31 December 2015. We assessed the rate of indeterminate results of the QFT-GIT in 38 patients with systemic vasculitis prior to any corticosteroid or immunosuppressant therapy, compared with 40 non-vasculitis patients with biological inflammatory syndrome, and 310 non-immunosuppressed patients matched for gender and age. RESULTS: Indeterminate results due to no response to phytohaemagglutinin A were more frequent in vasculitis patients (21.1%) compared with non-vasculitis patients with biological inflammatory syndrome (7.5%) (Fisher's exact test: P = 0.11) and to anonymized controls (7%) (P = 0.009). Responses to phytohaemagglutinin A were significantly lower in vasculitis patients compared with other groups (Kruskal-Wallis test: P < 0.0001) and compared with non-vasculitis patients with biological inflammatory syndrome (P = 0.0015). The multivariable analysis identified as independent predictors of an indeterminate result of the QFT-GIT: the presence of systemic vasculitis (odds ratio 9.64 [1.14-81.3], P = 0.037) and a high neutrophil-to-lymphocyte ratio (odds ratio 1.70 [1.21-2.37], P = 0.002). One patient with an indeterminate result of QFT-GIT developed active tuberculosis after one year of corticosteroid therapy for giant cell arteritis. CONCLUSION: Our results question the reliability of QFT-GIT to rule out latent tuberculosis in vasculitis patients at diagnosis, prior to immunosuppressant therapy.

[Giant cell arteritis: Ischemic complications]. / Complications ischémiques de l'artérite à cellules géantes.

GCA ischemic complications occur generally in patients with a yet undiagnosed or uncontrolled disease. When disease control is fair, ischemic complications may be due mostly to atheromatosis. Ophtalmic complications are most frequent and are dominated by anterior ischemic optic neuropathy. Vasculitic strokes occur essentially in the vertebrobasilar arterial territory. Overt vasculitic coronary disease is exceptional. The diagnosis of upper and lower limbs ischemic complications benefit from advances in echography (halo sign) and positron emission tomography imaging. Treatment relies on corticosteroids (initially 1mg/kg prednisone or more, preceded by intravenous methylprednisolone gigadoses if necessary), the control of cardiovascular risk factors and antiplatelet drugs; heparin may be indicated for threatening limbs ischemia.

Clinical Characteristics and Outcomes of Ocular Cicatricial Pemphigoid: A Cohort Study and Literature Review.

PURPOSE: Ocular cicatricial pemphigoid (OCP) is a rare systemic autoimmune disease and a potentially blinding subepithelial blistering disorder. The purpose of this study was to describe the clinical spectrum of the disease and to assess the efficacy and safety of immunosuppressive agents in a cohort of patients with OCP. METHODS: We conducted a monocentric retrospective cross-sectional cohort study of all unselected consecutive patients diagnosed with progressive OCP. Ocular and extra ophthalmological involvement as well as histological findings were gathered. Other outcomes were exposures to immunosuppressive agents defined by the use of a particular treatment. For each exposure, success in controlling ocular inflammation was graded as a complete response, response, or failure. Relapses and adverse events (AE) were also recorded. RESULTS: Seventeen patients (34 affected eyes), 35% of whom were women, were included, with an age at diagnosis of 75 ± 11 years. Corneal involvement was diagnosed in 30 of 34 eyes, and 22 of 34 eyes had progressive fibrosing conjunctival involvement. Sixty-two exposures to immunosuppressive agents or biologics were recorded: dapsone, n = 26; mycophenolate mofetil, n = 6; azathioprine, n = 4; cyclophosphamide, n = 10; rituximab, n = 14; and intravenous immunoglobulin, n = 2. Rates of response and of complete response achievement during the first 3 months were 84% and 45%, respectively. Response rates were 100%, 100%, 86%, 85%, and 80% for intravenous immunoglobulin, mycophenolate mofetil, rituximab, dapsone, and cyclophosphamide, respectively. Thirteen percent of those drugs were discontinued because of an adverse event in 4 patients. CONCLUSIONS: This study describes the efficacy of immunosuppressants or biologics with an acceptable safety profile for OCP.