Primary antiphospholipid syndrome and necrotizing pancreatitis: a diagnostic challenge.

The objective of this study was to report an unusual case of primary antiphospholipid syndrome (APS)-associated severe necrotizing pancreatitis. Since the APS was first recognized in the 1980s, a number of manifestations of the disorder have been described. We report primary APS presenting as severe necrotizing pancreatitis. This is the first such case to date that fulfills the revised Sapporo classification criteria. A 38-year-old previously healthy woman presented with new-onset hypertensive emergency and acute kidney injury. She subsequently developed severe epigastric pain attributable to necrotizing pancreatitis and extensive splenic infarcts. Biopsies of both the pancreas and kidney revealed thrombotic microangiopathy. Her lupus anticoagulant was positive on both weeks 1 and 12 of her disease course. A diagnosis of primary APS was made. Despite 6 months of aggressive care, she died of sepsis.

Myth and reality: practical test system for the measurement of anti-DNA antibodies in the diagnosis of systemic lupus erythematosus (SLE).

The myth persists that only the labor intensive Farr radioimmunoassay and Crithidia luciliae immunofluorescence (CL-IFA) are systemic lupus erythematosus (SLE)-specific tests. We compared them to ELISA with bacteriophage lambda DNA (EL-dsDNA) and denatured calf thymus DNA (EL-ssDNA). By percentile ranking, the specificity cut-off level was set both out of clinical context (SOCC) on 100 blood bank donors, and in clinical context (SICC) on 100 patients with either rheumatoid arthritis or scleroderma (50/50). Clinical sensitivity was calculated on 100 random SLE patients. At 95% SICC, the sensitivity of Farr, CL-IFA, EL-dsDNA, and EL-ssDNA was similar (95%CI): 76% (66-84), 76% (66-84), 63% (53-72), and 75% (65-83), respectively; 87% of the patients were positive by at least one method and 55%by all methods. At 99% SICC, the sensitivity was also similar (95% CI): 57% (47-67), 47% (37-57), 58% (47-67), and 43% (33-53), respectively. The areas under ROC curve were similar (95% CI) when patients were used as controls for specificity. At 99% SOCC, EL-ssDNA identified 89% positive, 2 negative but positive by another method at 95% SICC, and 9 negative (i.e. 89/2/9), followed by CL-IFA (80/6/14), Farr (76/12/12), and EL-dsDNA (64/23/13). Thus, at relatively low cost and easy automation, under the same conditions of specificity, the two ELISA tests combined were at least as good, if not superior, to CL-IFA or Farr: they showed similar clinical sensitivity and also identified more patients with anti-DNA antibodies.

Pantoprazole and perinuclear antineutrophil cytoplasmic antibody-associated vasculitis.

Systemic vasculitis may, at times, be drug-induced and associated with antineutrophil cytoplasmic antibodies (ANCA). However, pantoprazole, a commonly used and well-tolerated proton pump inhibitor, has not previously been reported to cause ANCA-associated syndromes. We describe a patient who developed interstitial nephritis, cutaneous vasculitis, a perinuclear ANCA staining pattern (pANCA) on immunofluorescence, and anti-myeloperoxidase antibodies (MPO-ANCA) in association with pantoprazole. We review various immune-mediated syndromes reported in association with proton pump inhibitors, including one report of omeprazole associated with interstitial nephritis and the development of ANCA.

Vertebral deformities identified by vertebral fracture assessment: associations with clinical characteristics and bone mineral density.

Whether vertebral fractures identified on radiographs are painful or not, they are associated with increased morbidity and mortality. Vertebral fractures on X-rays correlate with low bone mineral density (BMD) at the spine and hip in addition to several clinical characteristics. Evidence suggests that vertebral deformities detected by X-ray and by vertebral fracture assessment (VFA) show good agreement. We examined the relationship between VFA-detected vertebral deformities and patient characteristics as well as BMD by analyzing the records of 432 patients who had undergone dual-energy X-ray absorptiometry (DXA) scans with VFA. Patients' demographic data and T-scores were obtained from patient questionnaires and DXA scans. We categorized vertebral deformities by type and severity. Patients with vertebral deformities were significantly older and more likely to report a history of fracture after childhood. Significantly more estrogen use was reported in patients without deformity. Those with deformities had significantly lower T-scores at the femoral neck and total hip but not at the spine. Increased severity and number of deformities correlated with lower T-scores at the total hip and femoral neck but not the spine. In conclusion, vertebral deformities detected by VFA, like those on X-ray, correlate with both clinical characteristics and reduced bone mass at the hip. These relationships, in addition to rapid performance, convenience, and minimal radiation exposure, indicate VFA-detected vertebral deformities are a valuable adjunct in identifying patients in need of additional evaluation and treatment.

Musculoskeletal manifestations of endocrine disorders.

PURPOSE OF REVIEW: Much of our education about endocrine disorders focuses on their diagnosis and treatment. Although the musculoskeletal manifestations of endocrine disorders are well documented, they are often overlooked. This review will discuss new developments regarding those rheumatic manifestations. RECENT FINDINGS: Diabetic research is investigating connective tissue alterations in hand syndromes. A recent review elucidated the natural history of diabetic muscle infarction. Research has identified factors that stimulate osteoblast activity in diffuse idiopathic skeletal hyperostosis and bone loss in diabetics. Accumulating evidence documents thyroid disease coexisting with connective tissue disorders. Reports document cases of vasculitis occurring after propylthiouracil treatment. Finally, data clarifies the effects of thyroid dysfunction, hyperparathyroidism, acromegaly and hypercortisolism on bone. SUMMARY: Current research mainly relates to the effects of endocrine disorders on bone. As we advance our understanding of mechanisms that lead to rheumatic disorders in endocrine disease, we will improve our ability to treat them.