Efficacy of Cyclosporine in the Induction and Maintenance of Remission in a Systemic Lupus Erythematosus Patient Presenting with Macrophage-Activating Syndrome.

Macrophage-activating syndrome (MAS) is a rare condition characterized by dysfunctional macrophage activation leading to overproduction of cytokines and phagocytosis of erythrocytes, leukocytes, and platelets. MAS is associated with infectious diseases, malignancies, and autoimmune rheumatic disorders. Herein, we present a 22-year-old Hispanic woman with SLE who was hospitalized because of a three-week history of fever, fatigue, polyarthralgia, nausea, and abdominal pain. Initial laboratories showed severe pancytopenia with marked elevation of liver enzymes and ferritin levels. Bone marrow biopsy revealed macrophages with engulfed erythrocytes consistent with MAS. The patient was treated with high-dose corticosteroids, intravenous immunoglobulins, and cyclosporine 3 mg/kg/day. She had a remarkable clinical response to this therapy. She was continued on cyclosporine, and prednisone dose was gradually decreased to 7.5 mg daily without experiencing recurrent disease. She remained in full clinical remission for 12 months. Our case, together with other reports, suggests that combination therapy with corticosteroids, immunoglobulins, and cyclosporine appears to be effective for patients with SLE-associated MAS. Furthermore, cyclosporine seems to be a good drug for maintenance of remission.

Clinical Manifestations Associated with Overweight/Obesity in Puerto Ricans with Fibromyalgia Syndrome.

Objective. To determine the clinical manifestations associated with overweight/obesity in Hispanics from Puerto Rico with fibromyalgia syndrome (FMS). Methods. A cross-sectional study was performed in 144 patients with FMS (per American College of Rheumatology (ACR) classification criteria). Sociodemographic features, FMS-related symptoms, tender points (per ACR criteria), comorbidities, and FMS treatment were examined. BMI was calculated and patients were grouped into two categories: BMI ≤ 24.9 kg/m(2) (nonoverweight/obese) and BMI ≥ 25 kg/m(2) (overweight/obese). Bivariate and multivariate analyses were used to evaluate differences between the study groups. Results. The mean (standard deviation (SD)) age of patients was 50.2 (9.9) years; 95.1% were females and 75.7% were overweight/obese. In the bivariate analysis, overweight/obese patients were more likely to have self-reported memory impairment, anxiety, shortness of breath, and urinary frequency than nonoverweight/obese patients. In addition, the tender point count was higher in the overweight/obese group. In the logistic regression analyses, self-reported memory impairment and urinary frequency differences remained significant after adjusting for confounding variables. Conclusion. In this population of Puerto Ricans with FMS, overweight/obese patients experienced more FMS-related manifestations than nonoverweight/obese individuals. However, prospective studies are needed to confirm these associations and to elucidate if weight reduction interventions could favorably impact the severity of FMS.

Factors associated with tender point count in Puerto Ricans with fibromyalgia syndrome.

OBJECTIVE: To examine the factors associated with fibromyalgia syndrome (FMS) tender point count (TPC) in a group of Hispanic patients from Puerto Rico. METHODS: A cross-sectional study was performed in 144 FMS patients as determined using American College of Rheumatology [ACR] classification). Sociodemographic features, clinical manifestations, comorbidities, and pharmacologic agents were determined during the study visit. Tender points were assessed as described in the ACR classification for FMS. A t-test and one-way ANOVA test were used to examine the relationships between continuous, dichotomous, and nominal variables. RESULTS: The mean (standard deviation, [SD]) age of the FMS patients in this study was 50.2 (9.9) years; 95.1% were females. The mean (SD) TPC was 15.0 (4.7). Dysmenorrhea, the sicca syndrome, subjective swelling, increased urinary frequency, shortness of breath, headache, constipation, paresthesia, cognitive dysfunction, arthralgia, tiredness, morning stiffness, depression, and anxiety were associated with higher TPC. No associations were seen between socio-demographic features and FMS pharmacologic therapies. CONCLUSION: In this group of Puerto Ricans with FMS, TPC was associated with several FMS symptoms and comorbidities. This study suggests that TPC may be a simple and effective tool for assessing disease severity in FMS patients.

Thrombosed aneurysm as the initial manifestation of Takayasu arteritis.

Takayasu arteritis (TA) is a large-vessel vasculitis characterised by stenosis, dilation and/or aneurysm formation. We present a case of a 43-year-old man with an initial manifestation of an acute thrombosed aneurysm. He was found to have a thrombosed right common femoral artery aneurysm on Doppler ultrasound. Physical examinations revealed a substantial difference in blood pressure level between bilateral upper extremities, and absent pulses at right upper and lower extremities. The diagnosis was confirmed by angiography, which revealed 100% occlusion of the right common femoral artery. Biopsy of the thrombosed aneurysm was consistent with a diffuse lymphocytic subendothelial infiltrate. The patient was treated with high-dose corticosteroids and had a good response to treatment, as demonstrated by a decrease in sedimentation rate, and recovery of the right side pulses. This case helps to create awareness among physicians that TA may present with an acute occlusion of an aneurysm without the typical warning symptoms of TA.

Impact of managed care health insurance system for indigent patients with rheumatoid arthritis in Puerto Rico.

The aim of this study was to determine the clinical outcome among indigent patients with rheumatoid arthritis (RA) in Puerto Rico receiving their healthcare in a managed care system, as compared with non-indigent patients treated in fee-for-service settings. A cross-sectional study was conducted in 214 Puerto Ricans with RA (per American College of Rheumatology classification criteria). Demographic features, health-related behaviors, cumulative clinical manifestations, disease activity (per disease activity score 28), comorbid conditions, functional status (per Health Assessment Questionnaire), and pharmacologic profile were determined. Data were examined using uni- and multivariable (logistic regression) analyses. The mean (standard deviation (SD)) age of the study population was 56.6 (13.5) years; 180 (84.1 %) were women. The mean (SD) disease duration was 10.8 (9.6) years. Sixty-seven patients were treated in the managed care setting, and 147 patients received their healthcare in fee-for-service settings. In the multivariable analyses, RA patients treated in the managed care setting had more joint deformities, extra-articular manifestations, arterial hypertension, type 2 diabetes mellitus, cardiovascular events, fibromyalgia syndrome, and poorer functional status while having a lower exposure to biological agents than those treated in fee-for-service settings. Efforts should be undertaken to curtail the gap of health disparities among these Hispanic patients in order to improve their long-term outcomes.

Euforia-induced acute hepatitis in a patient with scleroderma.

Euforia, a supplement containing a variety of natural ingredients, is widely used as an antioxidant and anti-inflammatory formula. It is not approved by the US Food and Drug Administration and its side effects are unknown. We report a 45-year-old woman with limited systemic sclerosis who presented with jaundice and marked elevation of serum transaminases. One month before, she started taking Euforia juice. A liver biopsy disclosed submassive hepatocellular necrosis with histopathological changes consistent with toxic hepatitis. The patient's symptoms resolved with cessation of Euforia. Six months later, she persisted with abnormal liver function tests, but these resolved 18 months after discontinuation of Euforia. The mechanism by which Euforia causes liver injury is unknown. Some ingredients contained in this supplement (green tea, Aloe vera, noni and goji) are linked to hepatic injury. To our knowledge, this is the first report of hepatotoxicity associated with Euforia.

Lymphocytic mastitis preceding Sjögren's syndrome.

Sjögren's syndrome (SS) is a chronic autoimmune disease characterized by lymphocytic infiltration of exocrine glands and B cell hyperreactivity. Lacrimal and salivary glands are the most commonly involved causing keratoconjunctivitis sicca and xerostomia. A wide variety of other glandular and extraglandular manifestations can occur in SS. Lymphocytic mastitis is a rare presentation of several conditions including diabetes mellitus and autoimmune disorders. We report a case of a 43-year-old woman with a four-year history of arthralgias and positive antinuclear antibodies who developed a right painless breast mass. Biopsy revealed lymphocytic mastitis with predominant B cells. One year later she developed severe constitutional symptoms, sicca symptoms, lymphadenopathy, anemia, and interstitial lung disease. Serologies and minor salivary gland were consistent with the diagnosis of SS. This case further supports the association of lymphocytic mastitis with autoimmune diseases and demonstrates that it can even precede the clinical diagnosis of these entities.

Retrospective review of the clinical manifestations and outcomes in Puerto Ricans with idiopathic inflammatory myopathies.

BACKGROUND: The idiopathic inflammatory myopathies (IIM) are a heterogeneous group of conditions characterized by chronic inflammation of muscles, resulting in skeletal muscle weakness. Racial differences are apparent in the clinical manifestations and outcome of IIM. No previous studies have been conducted to evaluate the clinical manifestations and outcome of Puerto Ricans with IIM. OBJECTIVE: The objective of this study was to describe demographic characteristics, clinical features, and functional outcome of Puerto Rican patients with IIM. METHODS: Medical records at a university medical center were reviewed retrospectively to collect data regarding demographic factors, initial presentation, diagnostic tests, serologic markers, treatment, and disease course. A Functional Grading Scale was administered to 40 of the patients with IIM to determine their functional status. RESULTS: Fifty patients with IIM were identified. Eight patients had polymyositis (PM), 23 patients had dermatomyositis (DM), 2 patients had DM/PM with coexistent malignancy, 12 patients had juvenile DM, and 5 patients had DM/PM with an associated other rheumatologic disease. At diagnosis, proximal muscle weakness was present in all patients, 15 (30%) had myalgias, 5 (10%) patients had dysphagia, and 87% had elevation of serum muscle enzymes. Visceral involvement was rare. Interstitial fibrosis was identified in only 2 patients (1 juvenile DM and 1 PM). Twenty-six patients (51%) achieved complete remission. The mean score for the Functional Grading Scale was 28.6 (maximum 30). The lowest scores were seen for patients with disease duration of less than 2 years. CONCLUSION: Puerto Ricans with IIM in this survey showed a low presence of visceral involvement, high remission rate, and low mortality.