Pre-existing Systemic Lupus Erythematosus Research Articles

Primena nivolumaba kod bolesnice sa mukozalnim melanomom i sistemskim lupusom - prikaz slučaja sa pregledom literature

Introduction. Systemic lupus erythematosus (SLE) represents a multisystemic disease characterized by antibody production, complement activation, and immune complexes deposition. Certain types of malignancies occur more often, and conversely, some of them occur less often in SLE patients. Mucosal melanoma of the anorectal region represents a rare form of melanoma occurring in 1.5% of all melanoma patients, predominantly female. The introduction of novel agents dramatically changed the outcome in melanoma patients and introduced different adverse events, diverse contraindications, and drug interactions. Immune checkpoint inhibitors have a role in the maintenance of immunologic homeostasis. Patients with underlying autoimmune diseases were often excluded from clinical trials, for fear of possible autoimmune disease exacerbation or high-grade immune-related adverse events. Due to that, data regarding this subgroup of patients is limited, with no clear recommendations. Given the fact that prevalence among the general population is high (5-10%), autoimmune diseases represent common comorbidity in cancer patients. Having that in mind, it is of utmost importance to personalize the approach and individualize the SLE treatment and enable the use of PD-1 antibody in the safest and most useful way while keeping the SLE in control. Case report. Herein we present a 79-year-old with primary mucosal melanoma of the anorectal region, with lung metastasis and preexisting SLE in remission. Hydroxychloroquine was the only treatment for SLE. Nivolumab treatment was initiated in the standard dosing schedule. After the first and second follow-up, no further progression of melanoma was detected, with no SLE exacerbation and immune-related adverse events. Conclusion. PD-1 treatment in a patient with an underlying autoimmune disease represents a viable choice with a necessity for a multidisciplinary approach and close monitoring.

Clinical features of new-onset systemic lupus erythematosus during pregnancy in Central China: a retrospective study of 68 pregnancies.

The aim of this study is to explore the clinical features and pregnancy outcomes of Chinese patients with new-onset systemic lupus erythematosus (SLE) during pregnancy or puerperium. We retrospectively evaluated the data of all pregnant women with SLE managed at the First Affiliated Hospital of Zhengzhou University between April 2013 and March 2017. Clinical characteristics, laboratory features, medication use, and pregnancy outcomes were compared between pregnant women with new-onset SLE and pregnant women with pre-existing SLE. Risk factors for adverse pregnancy outcomes were determined using binary regression analyses. Overall, 223 pregnancies in 216 patients were included; 148 (69.6%) patients had a history of SLE, and 68 (30.4%) were diagnosed with SLE during pregnancy or puerperium. Most cases of new-onset SLE (72.1%) occurred during the first and second trimesters. Thrombocytopaenia (especially severe thrombocytopaenia) (76.5% vs 54.2%, P = 0.008; 39.7% vs 15.5%, P = 0.001) and anaemia (especially moderate anaemia) (73.5% vs 56.9%, P = 0.007; 52.9% vs 35.2%, P = 0.035) were more common in women with new-onset SLE than in women with pre-existing SLE and active disease during pregnancy. Additionally, patients with new-onset SLE experienced higher rates of moderate-to-severe disease activity than patients with pre-existing SLE (P < 0.01); disease activity occurred mostly during the first and second trimesters (75.4%). Compared with pre-existing SLE patients, disease activity in new-onset SLE patients occurred mostly in the first trimester (33.3% vs 15.3%, P = 0.043) and less in the third trimester (21.1% vs 47.2%, P < 0.001). Pregnancy loss was significantly higher in patients with new-onset SLE than in patients with pre-existing SLE (62.4% vs 27.1%, P < 0.001), with most cases occurring during the first and second trimesters (95.3%). However, there were no significant differences in neonatal outcomes between new-onset and pre-existing SLE patients with active disease. Within the new-onset SLE group, active disease was an independent risk factor for pregnancy loss (odds ratio [OR] = 16.185, confidence interval [CI] = 1.895-138.232, P = 0.011), whereas disease onset at late gestation was a protective factor against pregnancy loss (OR = 0.589, CI = 0.435-0.796, P = 0.013). Patients with new-onset SLE suffered greater haematological involvement (mainly thrombocytopaenia and anaemia) and higher rates of moderate-to-severe disease activity and pregnancy loss than patients with pre-existing SLE. Controlling disease activity and extending gestational age may improve pregnancy outcomes in women with new-onset SLE. Key Points • The clinical features of new-onset SLE during pregnancy and its impact on pregnancy outcomes have rarely been reported, especially in Chinese patients. • New-onset SLE during pregnancy in Chinese women occurred primarily during the first and second trimesters and was characterised by haematological disorders, including thrombocytopaenia and anaemia. • Women with new-onset SLE during pregnancy had significantly higher disease activity scores and pregnancy loss rates than women with pre-existing SLE, especially during the first and second trimesters; controlling disease activity and prolonging gestational age may improve pregnancy outcomes in this setting.

A Tale of Two Valves: Bioprosthetic Aortic Valve Obstruction in Systemic Lupus Erythematosus and Antiphospholipid Antibody Syndrome

Patients with systemic lupus erythematosus (SLE) and antiphospholipid antibody syndrome (APLAS) are at risk for cardiac manifestations, specifically valvular heart disease requiring valve replacement. Bioprosthetic valve endocarditis is an important cause of valve failure, and it is important to keep a wide differential, especially in patients with preexisting SLE and APLAS. In this E-challenge, 2 cases of bioprosthetic aortic valve endocarditis are presented; 1 case describes infective bacterial endocarditis on an aortic prosthesis and the second describes a patient with SLE and APLAS who developed bioprosthetic valve obstruction secondary to vegetations, consistent with nonbacterial endocarditis and thrombus. Etiologies for bioprosthetic valve obstruction and evaluation by echocardiography are explored. The comparison between these 2 cases specifically highlights the importance of keeping a wide differential in endocarditis, prosthetic valve vegetations, and bioprosthetic valve obstruction.

Photoprotective habits in children with systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a complex autoimmune disease that can involve multiple organ systems. Exposure to ultraviolet radiation (UVR) can exacerbate pre-existing SLE, and can even induce systemic manifestations. This study aimed to investigate the photoprotective habits of children with SLE and the factors that significantly influence those photoprotective habits. This questionnaire-based cross-sectional study included paediatric SLE patients being treated at the Department of Paediatrics at Siriraj Hospital, Mahidol University, between September 2018 and September 2019. Data were obtained from medical records and a face-to-face interview. Ninety-six patients were enrolled, with a female-to-male ratio of 8:1. The mean age of patients at enrollment was 13.7 ± 2.4 years. Of the 96 patients, 70 (72.9%) reported being directly exposed to sunlight for less than two hours per day, but 39% of patients spent time in the sun during the peak hours of UVR. Up to 95% of patients used sunscreen. However, only 64% of patients applied it every day, and only 35% of patients used an adequate amount of sunscreen. Girls were significantly more likely to apply sunscreen every day than boys were (p = 0.041). SLE patients with a shorter disease duration had significantly greater exposure to sunlight than patients with a disease duration of more than four years (p = 0.040). Sunscreen was the most common photoprotective method. However, most patients used sunscreen inappropriately. A shorter disease duration was significantly associated with more sunlight exposure. Regular evaluation and emphasis of the importance of photoprotection should be encouraged among paediatric SLE.

Maternal and Fetal Outcomes in Iranian Patients with Systemic Lupus Erythematosus: A Five-Year Retrospective Study of 60 Pregnancies.

As a multisystem autoimmune disease, Systemic Lupus Erythematosus (SLE) mainly affects women during reproductive age. This retrospective study was designed to investigate the fetal and maternal outcomes of Iranian women with SLE. Clinical and laboratory records of 60 pregnancies in 55 SLE patients who attended Hafez hospital, a tertiary referral center for high risk pregnancies and SLE patients affiliated with Shiraz University of Medical Science, were reviewed during April 2012 and March 2016. The mean age of the patients was 29.28±4.6 years and mean disease duration was 5.09±4.2 years. Live birth rate was 83.3% after exclusion of elective abortions. There were 50 live births, 3 neonatal deaths, 3 spontaneous abortions and 7 stillbirths. 9 (15%) women developed preeclampsia and there was 1 (1.6%) case of HELLP syndrome. Lupus flares occurred in 27 (45%) patients during pregnancy. Preterm delivery occurred in 11.6% of pregnancies. Skin and joints were the most frequently affected organs. Patients with previous lupus nephritis (n=18) were associated with a higher risk of maternal complication, but fetal outcomes were similar in both groups. Cesarean rate was about 66%, mostly related to fetal indications (50%). Pregnancies in most women with pre-existing SLE can now be managed with successful results although presence of previous lupus nephritis is still a major risk factor for adverse maternal outcomes. In our study, fetal outcome was not different between patients with lupus nephritis compared with the patients without nephritis who were under treatments. Hence, to achieve favorable long-term results, we recommend regular multispecialty treatment approaches and progestational counseling for women with SLE.

Intractable progressive pneumocephalus due to tuberculous meningoencephalitis

A 25-year-old woman with preexisting systemic lupus erythematosus (SLE) was admitted to the hospital with fever and general fatigue. CSF examination revealed pleocytosis (40/mm3), high protein (120 mg/dL), and normal adenosine deaminase levels. Despite treatment with antiviral, antibiotic, and antituberculous medications, her mentation deteriorated with severe brain swelling and …

Estrogen and Autoimmune Disease

Estrogenic hormones possess both immunostimulating and immunosuppressive properties. In systemic lupus erythematosus (SLE), pregnancy is associated with disease flares. In some situations, exogenous estrogen predisposes to development of new SLE, flares of preexisting SLE, and thromboses in susceptible individuals. In contrast, treatment with exogenous estrogen protects postmenopausal rheumatoid arthritis (RA) patients from active RA and osteoporosis. The search for estrogen-like compounds with anti-inflammatory properties may expand treatment options in RA.

A case of duplicitous diplococci

Disseminated gonococcal infection (DGI) is an uncommon complication of acute gonorrhoea that occurs more frequently in women than in men. Inherited complement deficiency or episodic complement deficiency in association with...

Cutaneous manifestations in relation to immunologic parameters in a cohort of primary myelodysplastic syndrome patients

Cutaneous lesions in myelodysplastic syndrome (MDS) may be specific or not and may reveal bone marrow transformation. Our purpose was to investigate in a cohort of 84 MDS patients the correlation of cutaneous findings with immunologic parameters and prognostic features of MDS in order to clarify their potential clinical significance. We studied a cohort of 84 newly diagnosed MDS patients in order to assess the cutaneous findings present at the time of diagnosis and during 1 to 3 years of follow-up. We described the clinical variety of cutaneous findings ascertained by histology. We also looked for any association between the group of MDS patients with skin manifestations and MDS subtype, immunologic and prognostic features highlighting transformation to acute leukaemia. Twenty-one patients presented cutaneous manifestations: 1 patient developed leukaemia cutis, 6 patients photosensitivity not associated with autoimmune disease, 3 prurigo nodularis, 2 Sweet's syndrome, 6 leucocytoclastic vasculitis, 2 ecchymoses and purpura associated with preexisting relapsing polychondritis, 1 patient subcutaneous nodules associated with Wegener's granulomatosis and 1 patient with malar rash and oral ulcers associated with preexisting systemic lupus erythematosus. Adjusted for age and gender, the presence of skin findings constitutes a significant predictor of the high-risk MDS subgroup (odds ratio, 3.59; 95% confidence interval, 1.18-10.92). Hypergammaglobulinemia was significantly higher in the MDS subgroup with skin manifestations (P = 0.03). Most MDS patients with cutaneous manifestations belong to the high-risk MDS subgroup and present hypergammaglobulinemia. Early biopsy of skin lesions in myelodysplasia is indicated.

Influence of maternal systemic lupus erythematosus on first‐trimester combined screening for chromosomal abnormalities

To explore the effect of maternal systemic lupus erythematosus (SLE) on first-trimester screening markers for Down syndrome. A retrospective study was conducted on 1150 normal singleton fetuses that underwent first-trimester combined screening for Down syndrome. Fetal delta nuchal translucency (NT), maternal serum PAPP-A and free beta-hCG were compared between pregnancies with SLE (n = 10) and without preexisting maternal disease (n = 1140). The medians +/- SD for delta NT, log(10) MoM of PAPP-A and free beta-hCG +/- SD in pregnancies with SLE and without maternal disease were - 0.18 +/- 0.29 versus - 0.18 +/- 0.33, 0.005 +/- 0.32 versus 0.02 +/- 0.26, and 0.22 +/- 0.19 versus - 0.014 +/- 0.28, with a p value of 0.7, 0.98 and 0.03, respectively. Patients with preexisting SLE have increased maternal serum-free beta-hCG levels in the first-trimester. But, because of the multimodal procedure of risk calculation there is no significant difference in the screen-positive rate after the combined first-trimester screening for trisomy 21.

Reversible Posterior Leukoencephalopathy in Patients with Systemic Lupus Erythematosus

The development of central nervous system (CNS) symptoms in patients with preexisting systemic lupus erythematosus (SLE) evokes a wide differential diagnosis. Reversible posterior leukoencephalopathy (RPLE) is a rapidly evolving neurologic syndrome with characteristic clinical and radiographic features. Conditions commonly associated with RPLE include hypertensive encephalopathy, eclampsia, immunosuppressive drugs, and inflammatory disorders. To describe our experience with RPLE in patients with concomitant SLE and review the literature. The details of 5 novel cases and a MEDLINE review of the literature concerning the development of RPLE in association with SLE are presented. All cases included patients with SLE who developed the acute onset of headache, altered mental status, visual changes, and seizures. Neuroimaging demonstrated posterior white matter edema involving the parietal, temporal, and occipital lobes. Complete clinical and radiographic recovery occurred with prompt antihypertensive treatment and supportive care. Literature review identified 16 additional cases of RPLE occurring in patients with active SLE; the majority of these reports was similar in presentation and outcome to our experience. It is likely that the clinical manifestations and neuroimages in these lupus patients were the result of the RPLE syndrome. Fortunately, this cause of "secondary" CNS symptoms in patients with SLE is readily reversible when diagnosed early and treated with blood pressure control and supportive care.

Underlying complement deficiency in patients with disseminated gonococcal infection.

The complement system was evaluated in 22 individuals with disseminated gonococcal infection. Three of the 22 patients exhibited a total serum complement activity that was greater than 2 SD below the normal mean. Of these three, one had a complete deficiency of C1r, a second patient had pre-existing systemic lupus erythematosus with low levels of C4, and the third had a C8 concentration that was 60% of normal. We conclude that the prevalence of inherited or acquired complement deficiency among patients with disseminated gonococcal infection exceeds that among the general population and is an important host factor predisposing to systemic infection with Neisseria gonorrhoeae.