Ann L. Parke

The thrombotic diathesis associated with the presence of phospholipid antibodies may be due to low levels of free protein S

PURPOSE To determine if abnormalities in the protein C/protein S anticoagulant system exist in patients with phospholipid antibodies who had the primary clinical complaint of fetal wastage. PATIENTS AND METHODS Eleven patients with fetal wastage and phospholipid antibodies were selected for study. Some patients also gave a history of previous thrombotic events related to oral contraceptives and/or pregnancy, but patients were not selected because of a history of clinical thrombosis. The levels of protein C (chromogenic assay), protein S (both free and bound) (Laurell rocket), and C4b-binding protein (Laurell rocket) were measured, and assays for the presence of antibodies against protein S or protein C were performed. RESULTS Seven of the 11 patients were found to have low levels of free protein S. Total protein S and protein C levels were within the normal range in all patients. Antibodies to protein C and protein S were not found in any patient. These findings suggest that free protein S levels may be abnormally low in some patients with phospholipid antibodies. CONCLUSION Free protein S levels are abnormally low in some patients with phospholipid antibodies, and this abnormality may be a factor contributing to the thrombotic diathesis associated with phospholipid antibodies.

Intravenous Gamma-Globulin, Antiphospholipid Antibodies, and Pregnancy

Excerpt To the Editor:In their letter ( 1 ) on the use of intravenous immunoglobulin therapy (IVIG) in pregnant women with antiphospholipid antibodies and a history of fetal loss, Francois and coll...

Antimalarial drugs and pregnancy

The use of chloroquine and hydroxychloroquine during pregnancy is controversial. These 4-aminoquinoline drugs cross the placenta and have been reported to cause fetal abnormalities, including loss of vision, ototoxicity, and cochleovestibular dysfunction. However, other reports suggest that 4-aminoquinoline drugs can be taken safely during pregnancy, even in dosages used to control systemic connective tissue diseases. We retrospectively studied eight patients who took antimalarial drugs continuously throughout pregnancy. There was a total of 14 pregnancies in women receiving these drugs; three occurred during periods of disease activity and all of these resulted in failure to complete pregnancy successfully. Of the remaining 11 pregnancies exposed to antimalarial drugs, one resulted in stillbirth; four resulted in spontaneous abortion; and six had normal full-term standard deliveries. None of the live births demonstrated any congenital abnormalities. Hence, it is possible for women to produce normal live babies despite taking large doses of these drugs. If patients with systemic lupus erythematosus are already taking antimalarial drugs when they become pregnant, it is believed that the risk of disease flare and loss of pregnancy from discontinuing therapy outweighs any risks to fetuses from continuing the medication.

Development and validation of the Lupus Impact Tracker: a patient-completed tool for clinical practice to assess and monitor the impact of systemic lupus erythematosus.

To derive and validate a brief patient‐completed instrument, the Lupus Impact Tracker (LIT), to assess and monitor the impact of systemic lupus erythematosus (SLE).

Hepatic disease, the gastrointestinal tract, and rheumatic disease.

In previous years, this review concentrated on the relationship between pathology in the gastrointestinal tract and rheumatologic complaints associated with this pathology. This year, we have emphasized the relationship between hepatic disorders and rheumatologic complaints, although a resumé of recent literature pertaining to the gastrointestinal tract and its rheumatologic consequences is also presented. We believe it is necessary to divert our primary focus of attention because of recent developments in identifying the extrahepatic effects of hepatitis C infection and the current interest in abnormalities of drug metabolism in various rheumatic and autoimmune disorders. These recent developments bring us full circle in incriminating not only bacterial and dietary antigens in the pathogenesis of the spondyloarthropathies but also viruses and exogenous chemicals as potential etiologic agents in genetically predisposed hosts, resulting in the development of a variety of diseases, including glomerulonephritis, vasculitis, Sjögrens syndrome, and systemic lupus erythematosus.

Focal Segmental Glomerulosclerosis Associated Seronegative Antiphospholipid Syndrome

Secondary Focal Segmental Glomerulosclerosis (FSGS) from thrombotic microangiopathies including Antiphospholipid Antibody Syndrome (APS), is well documented. We present a case with clinical features of APS but consistently negative serologies, suggesting ‘Seronegative APS (SNAPS)’. The patient was evaluated at the Division of Nephrology, University of Connecticut Health Center for progressive Chronic Kidney Disease (CKD). A renal biopsy exhibited thrombotic microangiopathy and associated FSGS. Systemic thrombophilia can be primary or secondary and has an extensive list of differential diagnoses. Distinct clinical features and serologic markers characterize a particular etiology. Antiphospholipid Syndrome (APS) is the most common acquired thrombophilia. Serologic evidence of APS is the presence of commonly recognized antibodies to phospholipids in this syndrome i.e. anticardiolipin (aCL) antibodies, Lupus Anticoagulant (LA) and β2-glycoprotein 1 (β2GPI) antibodies. Rarely a patient with classic clinical features of APS does not exhibit any of the above antibodies, suggesting ‘Seronegative APS (SNAPS)’.

57 – Sjögren's Syndrome

SjOgrens syndrome has been defined as a triad of complaints: keratoconjunctivitis sicca (dry eyes), xerostomia (dry mouth), and the presence of a well-defined connective tissue disease such as systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), or systemic sclerosis. As with other connective tissue diseases, this syndrome has a predilection for women, but it occurs more commonly in postmenopausal women, unlike SLE, which occurs most frequently during the childbearing years. Patients with other connective tissue diseases usually develop their SjOgrens complaints secondary to their initial disease. Reluctance to treat the inflammatory process has been the standard practice for many years. Replacing or stimulating glandular secretions is simply treating the end result of glandular destruction. Prevention of end organ damage should be the aim, just as it is in patients with SLE and RA. Because SjOgrens syndrome is an autoimmune disease that may predispose patients to malignant change, it could be argued that SjOgrens patients. With the known progression to malignancy and the chronic debility produced by inflammation and end organ damage, attempts to control the inflammatory process should be a priority.