Haematological manifestations in systemic lupus erythematosus (SLE) are frequently observed. They are diverse and range from mild to severe. Therefore, different treatment approaches are needed from simply keeping vigilant to significant immunosuppression. Most treatment evidence is based on case-reports or small retrospective studies, as few randomized controlled trials have been performed. The development of biological therapy has opened new possible ways to treat the most severe cases but further clinical trials are necessary. In this review we consider the most common and characteristic haematological manifestations of SLE patients, focusing on their pathogenesis and management.
Systemic lupus erythematosus (SLE) is a complex autoimmune rheumatic disease with multiple presentations, whose management presents many challenges. Many disease modifying or immunosuppressive drugs have been used with limited success, especially in patients with more severe disease activity. Belimumab is the first drug to be approved specifically for the treatment of SLE in more than 50 years. By blocking the B-cell activating factor, it interferes in B-cell differentiation and survival. Here we consider the results of the clinical trials that led to its approval, as well as the post-hoc analyses, follow-up studies and the current trials.
Objectives Tuberculosis (TB) is the most common opportunistic infection and cause of mortality among people living with HIV, and it is possible that it may also influence the evolution of the HIV infection. We assessed the differences between HIV‐positive and ‐negative people infected with TB. Methods The present study is a cross‐sectional retrospective study by electronic record revision. We included patients admitted to a tertiary hospital with a diagnosis of TB between 2011 and 2016, comparing those with HIV coinfection with non‐HIV patients, according to demographic and clinical characteristics. Results This study included 591 patients, of whom 32% were HIV‐coinfected. HIV‐TB patients were younger, with a predominance of male gender. Considering TB risk factors, there was a higher prevalence of homelessness and intravenous drug use in the HIV group. In the non‐HIV group, direct contact with other patients with TB and immunosuppression were more prevalent. Relative to TB characteristics, the HIV‐coinfected group presents with a higher prevalence of disseminated disease and a higher occurrence of previous TB infection. Cancer was the most frequent cause of immunosuppression in the HIV group and the number testing positive for TB via microbiological culture was lower. Assessment of microbiological resistance and in‐hospital mortality showed similar numbers in both groups. Conclusions There are few papers comparing clinical course of TB between HIV‐infected and non‐infected patients. Our study differs from others in the literature as we focused on a country with middling incidence of TB and further characterized the differences between HIV‐infected and non‐infected patients which can contribute to the management of these patients.
We present a case report of a 42 year old female, diagnosed at the age of 3 with Juvenile Dermatomyositis. The clinical course was severe and refractory to immunosuppressive therapy. Currently, she is mostly affected by severe muscle atrophy, large joint contractures, calcinosis, and a lipodystrophy associated metabolic syndrome with hypertriglyceridemia, insulin resistance, high total testosterone and hepatic steatosis. She developed Hodgkin´s lymphoma in the course of her disease. Personalized therapeutic choices are discussed as regards juvenile dermatomyositis complications.
BackgroundPortugal has one of the highest prevalence and incidence of tuberculosis (TB) and latent TB (LTB) in Western Europe1. Screening and treatment is performed in Centers for Diagnosis of Chest Pathology (CDP), specifically designated by the Health Authority. Biological Therapy (BT) for Systemic Autoimmune Diseases (SAID) has been associated with an increased reactivation of TB2 and national3 and international4 guidelines recommend LTB screening before therapy onset, a practice that has been followed in our Unit since 2001, when the first patient started BT. The most recent American College of Rheumatology guidelines recommend that patients with negative baseline screening should be re-screened annually4.MethodsThe aim of our study was to capture and treat LTB infections in all patients undergoing current biological treatment for SAID in our Unit. We conducted a prospective study by sending the patients a letter of referral to the CDP, requesting TB re-screening, to be followed by therapy if required. The letter explained to the patient the need for referral and contained clinical information for the CDP. Six months later, medical records were reviewed and patients were contacted telephonically when clinical information was absent or insufficient. Screening intent involved the following: 52 patients with Rheumatoid Arthritis (RA), 26 with Ankylosing Spondylitis (AS), 16 with Psoriatic Arthritis (PA), 4 with Inflammatory Bowel Disease (IBD) and 2 Seronegative Spondyloarthropathies (SS). Demographic characteristics were as follows: RA: F:M=42:10, x̄ age 59 y, x̄ disease duration 12 y; x̄ BT for 4.9 y. AS:F:M=10:16, x̄ age 47 y, x̄ disease duration 11 y, x̄ BT for 5.5 y; PA: F:M=8;8, x̄ age 55 y, x̄ disease duration 14 y, x̄ BT for 6.9 y; IBD: F:M=3:1, x̄ age 47 y, x̄ disease duration 7.5 y and x̄ BT for 3.75 y. The SS group consisted of 2 females; x̄ age 51 y, x̄ disease duration 8.5 y, x̄ BT for 4 y. BT mostly consisted of infliximab, etanercept, adalimumab or tocilizumab; 12 RA patients were on Rituximab on demand. Before BT onset, 14/52 patients with RA, 4/26 with AS and 1/16 with PA had a history of treated tuberculosis.ResultsOnly 36 out of 100 patients were screened at the CDP as a result of our study: 23 received the letter but did not read it, 19 did not receive the letter for several reasons, 3 refused to schedule but 9 promised to schedule the appointment after the telephone contact, 8 already had their follow-up appointment at the CDP within the past year, 1 had a future appointment scheduled and 1 had to suspend the biological agent shortly after receiving the letter. Screening was performed in 9 patients with RA, 18 with AS, 6 with PA, 2 with IBD, 1 in the SS group. Overall, 16 of 36 screened patients required LTB therapy, interrupting BT/the next cycle in the first month of LTB treatment (3 with AR, 10 with AS, 2 with PA and 1 with IBD).ConclusionsLTB is an important problem in our patient population under BT. Our study emphasizes the need for systematic re-screening of these patients and the deve...
With the increased presence of the elderly in the emergency department, sepsis represents a major cause of death. The Sepsis Taskforce developed the quick SOFA (qSOFA) score which consists of only 3 items, is quick and easy to apply, and establishes a mortality risk for patients. A retrospective review of electronic records of elderly patients, admitted during a year, with an infectious disease was made. Outcomes were in-hospital mortality, admission to intensive care unit, need for invasive mechanical ventilation, non-invasive mechanical ventilation, antibiotic escalation, and length of stay. In the 310 patients, 24% had a positive qSOFA score at admission. It correlated with increased mortality (2.7 times), also increased ICU admission (3.5 times), greater need for invasive ventilation (risk ratio 4.4), non-invasive ventilation (3 times higher), and a longer length of stay (2.3 days). qSOFA was a good predictor of IMV and NIMV need, ICU admission, and death.
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