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BACKGROUND: Nephrotic syndrome caused by minimal mesangial lupus nephritis is considered rare. Nephrotic syndrome can be caused by minimal mesangial lupus nephritis with diffuse epithelial foot-process effacement and lupus podocytopathy. CASE REPORT: A 23-year-old Japanese woman diagnosed with mixed connective tissue disease was admitted because of weight gain and generalized edema for 2 weeks prior to admission. She had butterfly-shaped erythema on her cheeks, proteinuria, leukocytopenia with lymphocytopenia, and hypoalbuminemia. She was positive for antinuclear antibodies, and specific autoantibodies were only positive for the ribonucleoprotein (RNP) antigen. She was diagnosed with systemic lupus erythematosus. Renal biopsy showed minor glomerular abnormalities, and immunofluorescence revealed peripheral deposits of IgM and complement C3c. Electron microscopy revealed diffuse podocyte foot-process effacement of >80% of the capillary loop surfaces, with only a few subendothelial deposits. Consequently, we diagnosed minimal mesangial lupus nephritis with lupus podocytopathy. On hospital day 4, we administered 1000 mg/day of methylprednisolone for 3 days, followed by prednisolone 50 mg/day, but proteinuria persisted. On day 12, we administered tacrolimus (3 mg/day). Proteinuria improved and then disappeared on day 17. Prednisolone was gradually tapered and stopped after 3 years, although tacrolimus 3 mg/day was continued. No flare-up was observed 4 years after admission. CONCLUSIONS: Tacrolimus showed good efficacy in this case of minimal mesangial lupus nephritis with lupus podocytopathy. Prospective and randomized controlled trials should be conducted to demonstrate the efficacy of tacrolimus for this indication. [ABSTRACT FROM AUTHOR]

Objective To investigate the impact of medical and obstetric complications associated with mixed connective tissue disease (MCTD) in pregnancy. Method We analyzed 68 pregnancies from a systematic literature review and 12 pregnancies affected by MCTD at our centre between 1986 and 2015 for medical and obstetric complications. Results During pregnancy 37.1% had active MCTD and 26.7% had relapsed. Maternal complications included caesarean section (31.1%, n = 19), preeclampsia (17.6%, n = 13), thromboembolism events, and death (2.5%, n = 2 for each). Fetal complications included prematurity (48.1%, n = 25), intrauterine growth restriction (38.3%, n = 19), and neonatal lupus (28.6%, n = 18, including chondrodysplasia punctata). More than half (n = 10) of the neonatal lupus cases were explained by anti-U1RNP only. The perinatal mortality rate was 17.7% (n = 14). Pregnant women with active disease had higher rates of prematurity (OR = 7.60; 95%CI [1.93; 29.95]) and perinatal death (OR = 16.83; 95%CI [1.90; 147.70]). Conclusion MCTD in pregnancy puts women at risk of medical and obstetric complications, and disease activity probably increases this risk. [ABSTRACT FROM AUTHOR]

Objectives: Systemic lupus erythematous (SLE) is a chronic autoimmune disease that affects women primarily of childbearing age. The objective of this study was to determine the neonatal and maternal outcomes of pregnancies in SLE patients compared to pregnancies in healthy controls. Methods: We conducted a retrospective cohort study in a tertiary care hospital in Oman between January 2007 and December 2013. We analyzed 147 pregnancies and compared 56 (38.0%) pregnancies in women with SLE with 91 (61.9%) pregnancies in healthy control women. Disease activity was determined using the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI). Results: The mean age of the cohort was 30.0±5.0 years ranging from 19 to 44 years old. Patients with SLE were treated with hydroxychloroquine (n = 41; 73.2%), prednisolone (n = 38; 67.8%), and azathioprine (n = 17; 30.3%). There was no disease activity in 39.2% (n = 22) of patients while 41.0% (n = 23), 12.5% (n = 7), and 7.1% (n = 4) had mild (SLEDAI 1-5), moderate (SLEDAI 6-10), and severe (SLEDAI ≥ 11) disease activity, respectively, at onset of pregnancy. Pregnancies in patients with SLE were associated with higher abortions (42.8% vs. 15.3%; p < 0.001), gestational diabetes (28.3% vs. 10.2%; p = 0.004), polyhydramnios (7.1% vs. 0.0%; p = 0.020), previous preterm pregnancies (8.9% vs. 1.0%; p = 0.030), and intrauterine growth retardation (21.4% vs. 0.0%; p < 0.001) when compared to pregnancies in healthy control women. Furthermore, the neonates born to mothers with SLE were more likely to be preterm (28.5% vs. 1.0%; p < 0.001), have a low birth weight (< 2 500 g) (32.1% vs. 1.0%; p < 0.001), and were associated with stillbirth (7.1% vs. 0.0%; p = 0.010) when compared to neonates born to healthy control mothers. Conclusions: Pregnancies in women with SLE were associated with higher neonatal and maternal complications. Therefore, pregnant women with SLE should have their pregnancy accurately planned, monitored, and managed according to a multidisciplinary treatment schedule. [ABSTRACT FROM AUTHOR]