Faculty Bibliography

INTRODUCTION: IgG4-Related Disease is a newly recognized condition which is increasingly diagnosed by practitioners due to improvement in clinical awareness. Men and women have been found to be affected by this disease in various organs, more commonly with involvement of the salivary and lacrimal glands as well as pancreas and liver. Areas covered: The diagnosis and management of this condition remain challenging as biomarkers and therapies are being investigated. Hallmark features on histology are still the gold standard for confirmation of diagnosis, whereas serum IgG4 level has been shown to be neither necessary nor sufficient for the diagnosis. Glucocorticoids remain the most effective initial management for this condition while there are limited clinical trials on the effectiveness of maintenance therapy. Expert commentary: This review serves as an update on approaches for diagnosis and management of IgG4-RD. Most of the known data in this field comes from retrospective cohort studies and expert consensus guidelines but new ongoing prospective studies, clinical trials and better understanding of the pathogenesis of this condition are promising.

Norethindrone acetate/ethinyl estradiol (Estrostep; Warner Chilcott, Rockaway, New Jersey) is an "estrophasic" type of oral contraceptive, which combines a continuous low progestin dose with a gradually increasing estrogen dose. In clinical trials, this medication failed to produce clinically significant changes in serum lipid levels. We report a case of severe hypertriglyceridemia-induced acute pancreatitis in a 24-year-old woman caused by Estrostep, occurring nearly 10 years after she began using the drug. The patient was admitted to the medical intensive care unit (ICU) for aggressive volume resuscitation and management of severe electrolyte abnormalities. Laboratory studies obtained on admission indicated severe hypertriglyceridemia (2,200 mg/dL), hyponatremia (120 mEq/L), and hypocalcemia (0.78 mmol/L). Amylase and lipase levels were also elevated (193 and 200 U/L, respectively). Ranson score calculated after 48 hours of admission was 4, and her Acute Physiology and Chronic Health Evaluation (APACHE) IV score was 35. Treatment included an insulin infusion, omega-3 fatty acid esters, and gemfibrozil. The insulin infusion reduced serum triglyceride levels by 50% after 1 day of treatment and to 355 mg/dL by day 7 of her ICU course. We believe that this is the first reported case of severe, acute hypertriglyceridemia-induced pancreatitis caused by this medication.

Anti-P antibodies have been associated with organ involvement in SLE, such as in autoimmune hepatitis, and have been suggested to be directly pathogenic. Neuropsychiatric lupus, lupoid hepatitis, autoimmune hepatitis, lupus nephritis, and Chagas' disease have been associated with the presence of anti-P antibody. This review seeks to look into the current literature on anti-P antibody and the association between SLE and non-SLE autoimmune connective tissue disorder.