Transplacental transfer of maternal anti-Ro and/or anti-La autoantibodies can result in fetal cardiac disease including congenital heart block and cardiomyopathy, called cardiac Neonatal Lupus (NL). Thousands of women are faced with the risk of cardiac NL in their offspring, which is associated with significant morbidity and mortality. There are no known therapies to permanently reverse third degree heart block in NL, although several treatments have shown some effectiveness in incomplete heart block and disease beyond the atrioventricular node. Fluorinated steroids taken during pregnancy have shown benefit in these situations, although adverse effects may be concerning. Published data are discordant on the efficacy of fluorinated steroids in the prevention of mortality in cardiac NL. β-agonists have been used to increase fetal heart rates in utero. The endurance of β-agonist effect and its impact on mortality are in question, but when used in combination with other therapies, they may provide benefit. No controlled experiments regarding the use of plasmapheresis in cardiac NL have been performed, despite its theoretical benefits. Intravenous immunoglobulin was not shown to prevent cardiac NL at a dose of 400 mg/kg, although it has shown effectiveness in the treatment of associated cardiomyopathy both in utero and after birth. Retrospective studies have shown that hydroxychloroquine may prevent the recurrence of cardiac NL in families with a previously affected child, and a prospective open-label trial is currently recruiting patients in order to fully evaluate this relationship.
Mannoproteins on the cell wall of yeast and fungi help regulate cell shape, porosity, and cell-cell interactions, including those required for attachment to host cells by fungal pathogens. The mannose-containing oligosaccharides on proteins and lipids are extended in the Golgi by glycosyltransferases that use GDP-mannose as the sugar substrate. A membranebound transporter that, in Saccharomyces cerevisiae, is encoded by the VRG4 gene catalyses delivery of GDP-mannose into the lumen of the Golgi. We report here the cloning of the homologous VRG4 gene from the pathogenic yeast, Candida glabrata, by functional complementation of an S. cerevisiae vrg4 mutant. The sequence of the CgVrg4 protein displays significant homology to GDP-mannose transporters from other yeast, fungi, protozoa, and plants. CgVRG4 fully complements the glycosylation defect and other cell wall associated vrg4 mutant phenotypes. Like ScVRG4, CgVRG4 is essential for the viability of C. glabrata. These results suggest that, as in S. cerevisiae, CgVrg4p accounts for all of the GDP-mannose transport activity in the Golgi lumen. The GenBank Accession No. for CgVRG4 is #AF360395.
Haemophagocytic lymphohistiocytosis (HLH) is a potentially life-threatening syndrome caused by excessive immune activation. Secondary HLH has been described in autoimmune diseases. We detail the case of a 28-year-old African American woman who developed HLH in the setting of systemic lupus erythematosus with collapsing lupus podocytopathy superimposed on mesangial proliferative lupus nephritis class II. Genotyping for APOL1 risk alleles revealed the presence of double (G1/G2) risk alleles. Our patient achieved a complete renal recovery and resolution of HLH within 1 month of treatment with steroids and mycophenolate mofetil, highlighting the importance of prompt, aggressive therapy.
Cocaine adulterated levamisole is an increasingly reported cause of skin necrosis, arthralgia and systemic vasculitis, but renal involvement is uncommon. We present a case of a 40-year-old Hispanic man with a history of cocaine abuse who presented with acute kidney injury to the rheumatology clinic where he was being treated for chronic inflammatory arthritis. He was found to have a serum creatinine of 2.5 mg/dL, microscopic haematuria and subnephrotic proteinuria, along with positive proteinase 3, myeloperoxidase, anticardiolipin antibodies and an elevated antinuclear antibody titre. The renal pathology revealed focal necrotising glomerulonephritis with crescentic features and mild immune type deposition. The patient was treated with cocaine abstinence, pulse dose steroids followed by maintenance prednisone, rituximab and cyclophosphamide. His renal function subsequently improved but did not normalise. We believe that his incomplete improvement was due to the degree of kidney injury on presentation as well as recidivism with cocaine use.
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