Segmental arterial mediolysis: a rare non-inflammatory cause of mesenteric bleeding.

Visceral abdominal aneurysms can originate from multiple disease states, including inflammatory, non-inflammatory and infectious aetiologies. It is important to follow a stepwise approach to make the correct diagnosis, because disease prognosis and management can be substantially different. We describe a 60-year-old Caucasian woman who presented from an outside facility to our University Hospital in a critical state with abdominal bleeding. She had no findings to support a vasculitic process, nor a concern for infectious aetiologies. She required a thoughtful approach and detailed imaging to diagnose a rare non-inflammatory disease as the cause for her mesenteric bleeding-segmental arterial mediolysis (SAM). Through our case and discussion, we describe the importance of recognising this rare entity and of understanding how early recognition can save patients from significant morbidity and unnecessary potential harmful therapeutic options.

Bullous skin lesions in an adult male: a diagnostic dilemma.

BACKGROUND: Henoch-Schönlein Purpura (HSP) is an IgA small-vessel vasculitis that is primarily a disease of childhood. Its presentation in adulthood is rare and has a more severe disease course. We present a case with an atypical presentation of this disease that was a diagnostic challenge for multiple providers. CASE REPORT: A 42-year-old man noticed bullous lesions over his ankles that spread to his entire legs over a few weeks. They later became necrotic and ulcerated areas. His primary care physician and 2 dermatologists could not reach a definitive diagnosis. He then presented to our hospital with new abdominal pain, rectal bleeding, and a new elevation in liver enzymes. A biopsy of his skin lesions led to the diagnosis of HSP. CONCLUSIONS: We discuss this highly unusual initial presentation with bullous skin lesions and liver enzyme abnormalities and explore the medical literature to understand its pathogenesis. Clinicians need to be aware of this rare presentation to avoid a delay in diagnosis and management.

Adherence to medications in systemic lupus erythematosus.

BACKGROUND: Lack of adherence is a ubiquitous problem which can be a hindrance in the treatment of chronic conditions like systemic lupus erythematosus (SLE). OBJECTIVES: A random sample of 63 SLE patients attending rheumatology clinics associated with University Medical Centers were surveyed to measure level of adherence to their SLE medications and to identify the risk factors that have been associated previously with nonadherence to these medications. METHODS: Information on traditional SLE outcomes was obtained by face-to-face interviews and medical record review. Various patient proposed strategies were identified to improve adherence to these medications. RESULTS: When considering adherence estimates of > or =80% as representing sufficient adherence for achieving a therapeutic response, adherence to medications was only modestly adherent, likely limiting the effectiveness of the prescribed medication regimens. Based on pharmacy refill information 61% of the patients were sufficiently adherent to prednisone, 49% to hydroxychloroquine, and 57% to other immunosuppressant medications. Significant risk factors of insufficient adherence included being single, low educational level, presence of other comorbidities, limited comprehension of physician explanations and instructions, and having to take the medication more than one daily. Based on subject reports, busy life styles were among the most important barriers to adherence whereas pillboxes were considered most helpful for helping with medication adherence. CONCLUSION: Although lack of sufficient adherence to medications appears to be a multifactorial problem, improved communication between the healthcare provider and the patient, and less complicated medication regimens, may be especially suitable interventions to improve adherence to medications.

Effectively measuring adherence to medications for systemic lupus erythematosus in a clinical setting.

OBJECTIVE: To assess the reliability and concurrent validity of the Medication Adherence Self-report Inventory (MASRI) when used in systemic lupus erythematosus (SLE), to investigate the predictive validity of the MASRI using pharmacy refill information as the criterion standard, and to propose a sensible approach to the screening for nonadherence in a clinical setting. METHODS: Adherence to 2 medications (hydroxychloroquine and prednisone) was measured in 55 patients using the MASRI, pill counts, and physician ratings (MD scale). Adherence based on pharmacy refill information served as a criterion standard with nonadherence defined as adherence rates <80%. To determine test-rest reliability of the MASRI, 20 patients completed the measure twice within a 2-week period. RESULTS: Using pharmacy information, 39% of the patients were nonadherent to prednisone and 51% to hydroxychloroquine. The MASRI had acceptable internal consistency (Cronbach's alpha 0.7) and good reliability. Irrespective of the drug assessed, MASRI ratings were moderately correlated with patient adherence (pharmacy), supporting the concurrent validity of the MASRI. The combination of adherence estimation by MD scale rating at <85% and by MASRI at <90% was 87% sensitive and 86% specific for identifying patients who were nonadherent to prednisone. These cutoff values also appeared suitable for identifying nonadherence to hydroxychloroquine. CONCLUSION: The MASRI is a reliable measure of adherence to medications in SLE. The measure has concurrent and predictive validity. When combined with the MD scale, the MASRI appears to be a useful screening tool for nonadherence in patients with SLE that could be suitable for clinical practice.

Family study of fibromyalgia.

OBJECTIVE: To assess for familial aggregation of fibromyalgia (FM) and measures of tenderness and pain, and for familial coaggregation of FM and major mood disorder (major depressive disorder or bipolar disorder). METHODS: Probands meeting the American College of Rheumatology criteria for FM and control probands with rheumatoid arthritis (RA) and no lifetime diagnosis of FM were recruited from consecutive referrals to 2 community-based rheumatology practices. Probands were ages 40-55 years and had at least 1 first-degree relative age 18 years or older who was available for interview and examination. All probands and interviewed relatives underwent a dolorimeter tender point examination and a structured clinical interview. Interviewed relatives were asked about first-degree relatives who were not available for interview, using a structured family interview. Logistic and linear regression models, adjusting for the correlation of observation within families, were applied to study the aggregation and coaggregation effects. RESULTS: Information was collected for 533 relatives of 78 probands with FM and 272 relatives of 40 probands with RA. FM aggregated strongly in families: the odds ratio (OR) measuring the odds of FM in a relative of a proband with FM versus the odds of FM in a relative of a proband with RA was 8.5 (95% confidence interval [95% CI] 2.8-26, P = 0.0002). The number of tender points was significantly higher, and the total myalgic score was significantly lower in the relatives of probands with FM compared with the relatives of probands with RA. FM coaggregated significantly with major mood disorder: the OR measuring the odds of major mood disorder in a relative of a proband with FM versus the odds of major mood disorder in a relative of a proband with RA was 1.8 (95% CI 1.1-2.9, P = 0.013). CONCLUSION: FM and reduced pressure pain thresholds aggregate in families, and FM coaggregates with major mood disorder in families. These findings have important clinical and theoretical implications, including the possibility that genetic factors are involved in the etiology of FM and in pain sensitivity. In addition, mood disorders and FM may share some of these inherited factors.