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.

Association of the use of statins with disease activity and functional status in Puerto Ricans with rheumatoid arthritis.

OBJECTIVE: Statins, which appear to have anti-inflammatory and immunomodulatory effects, may benefit patients with rheumatoid arthritis (RA). Our study sought to determine the association of statins use with disease activity and functional status in a group of patients with RA. METHODS: A cross-sectional study was performed in 209 Puerto Ricans with RA (per the 1987 classification criteria of the American College of Rheumatology). Demographic features, lifestyle-related behaviors, disease activity (per Disease Activity Score 28), comorbid conditions, functional status (per Health Assessment Questionnaire), pharmacologic therapy, and patients' and physicians' global assessments using visual analogue scales, were determined. Data were examined using univariate, bivariate, and multiple logistic regression analyses. RESULTS: The mean (standard deviation [SD]) age of the study population at study visit was 56.8 (13.5) years (range: 24-86 years); 175 patients (83.7%) were women. The mean (SD) disease duration was 10.4 (9.5) years (range: 0.0-44.0 years). Thirty-two (15.3%) patients were using statins at study visit, and 36 (17.2%) had used statins in the past. In the multivariable analysis, the current use of statins was associated with higher functional status (odds ratio 0.42, 95% confidence interval 0.22-0.80) than was nonuse, after adjusting for age, disease duration, arterial hypertension, coronary artery disease, and dyslipidemia. No association between either current or past use of statins and disease activity was found. CONCLUSION: In this group of RA patients, the current use of statins was associated with a higher functional status; conversely, no association was found between statins use and disease activity. However, larger and longitudinal studies are required to confirm these findings.

Clinical and sociodemographic features of Puerto Ricans with systemic sclerosis.

INTRODUCTION: Systemic sclerosis is an autoimmune disease of unknown etiology characterized by fibrotic changes in the skin, blood vessels, and various internal organs. The disease has a wide spectrum of presentation and a variable clinical course that includes limited skin involvement to life-threatening disease. Clinical manifestations and disease severity differs among ethnic groups. The objective of this study is to describe the clinical and sociodemographic features of patients with well-characterized systemic sclerosis from Puerto Rico. METHODS: A structured questionnaire was completed for each patient to gather information about demographic factors, clinical manifestations, laboratory findings, diagnostic studies, and pharmacologic treatments. RESULTS: Of the 24 patients with systemic sclerosis, 96% were females, 83% had Raynaud's phenomenon, 67% had gastrointestinal involvement, 63% had skin hypopigmentation, 50% had digital pitting scars, 46% had arterial hypertension, 11% had pulmonary hypertension, and 4.8% had renal involvement. The overall median modified Rodnan skin score was 24.5 (inter-quartile range 16.0-31.3). Pulmonary function tests resulted in abnormal in 60% of 14 patients, of which 57% had restrictive lung disease (FVC < 70%) and 42.9% had decreased diffusion capacity. Serologically, 66.7% were positive for antinuclear antibody and 62.5% were positive for anti-centromere. CONCLUSIONS: In this study, the predominant clinical features of Puerto Ricans with systemic sclerosis were gastrointestinal involvement, Raynaud's phenomenon, digital pitting scars, and lung disease. Patients had a moderate severity of skin disease. The presence of renal involvement and pulmonary hypertension were low in our group. No significant differences were found between systemic sclerosis disease subsets.

Impact of age on clinical manifestations and outcome in Puerto Ricans with rheumatoid arthritis.

INTRODUCTION: Disease expression and outcomes in rheumatoid arthritis (RA) vary among different ethnic groups. There are limited data on the impact of age on disease severity and outcomes among Hispanics. Thus, we determined the demographic characteristics, clinical manifestations, comorbidities, pharmacologic profile, and functional status among Puerto Ricans with RA of different age groups. METHODS: A cross-sectional study was conducted in 214 Puerto Rican patients with RA (per American College of Rheumatology classification criteria). Demographic features, health-related behaviors, cumulative RA manifestations, treatment profiles, disease activity (Disease Activity Score 28), comorbid conditions, and functional status (Health Assessment Questionnaire) were determined at study visit. Three age groups were studied: <40, 40-59, and > or =60 years. Data were examined using univariable and multivariable (logistic regression) analyses. RESULTS: The mean (SD) age of the study population was 56.5 (13.6) years with a mean disease duration (SD) of 10.8 (9.7) years; 180 patients (84.1%) were women. In the multivariable analyses, patients aged > or =60 years were more likely to have joint deformities, extra-articular manifestations, and comorbidities such as dyslipidemia, arterial hypertension, diabetes mellitus, vascular events, osteoarthritis, low back pain, and osteoporosis. In addition, older patients used corticosteroids more frequently. No differences were found for the use of disease-modifying anti-rheumatic drugs or biologic agents. CONCLUSIONS: Puerto Rican RA patients aged > or =60 years present a severe type of disease having more joint damage, extra-articular manifestations, and comorbidities than younger patients. These disparities must be considered when establishing effective therapy for older RA patients.

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.

Incident arterial vascular events in a cohort of Puerto Ricans with rheumatoid arthritis.

OBJECTIVE: The increased morbidity and mortality associated with cardiovascular events in patients with rheumatoid arthritis has been linked to traditional and nontraditional factors. However, these factors vary among different ethnicities. Few studies have described these features in Hispanic populations. Thus, we determined the clinical correlates of arterial vascular events in Hispanics from Puerto Rico. METHODS: A cross-sectional study was performed in a cohort of 405 Puerto Ricans with rheumatoid arthritis. Demographic parameters, health-related behaviors, clinical manifestations, disease activity (per Disease Activity Score 28), functional status (per Health Assessment Questionnaire), comorbidities, and pharmacotherapy were compared in patients with and without incident arterial vascular events. The latter was defined as the occurrence of myocardial infarction, angina pectoris, vascular procedures for coronary artery disease, stroke, or peripheral artery disease. Study groups were analyzed using bivariate and multivariate analyses. RESULTS: Of the total study population, 87.2% were woman. The mean age at study visit was 56.1 ± 13.9 years, and the mean disease duration was 15.0 ± 13.2 years. Arterial vascular events occurred in 43 patients (10.6%). In the multivariate analysis adjusted for age and sex, arterial hypertension, dyslipidemia, metabolic syndrome, extra-articular manifestations, higher Health Assessment Questionnaire score, and number of hospitalizations were associated with arterial cardiovascular events. CONCLUSION: In this cohort of Puerto Ricans with rheumatoid arthritis, traditional and nontraditional factors, particularly extra-articular manifestations and functional disability, were associated with arterial vascular events. Awareness of these associations may help to implement clinical strategies in this group of rheumatoid arthritis patients at risk of arterial vascular events.

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.

Efficacy of Rituximab in a Systemic Lupus Erythematosus Patient Presenting with Diffuse Alveolar Hemorrhage.

Diffuse alveolar hemorrhage (DAH) is a life-threatening complication of systemic lupus erythematosus (SLE). Although infrequent, its mortality is very high. While there are no established therapeutic guidelines, DAH has been traditionally managed with high-dose intravenous (IV) corticosteroids, cyclophosphamide, and plasma exchange. The efficacy of alternative therapies such as rituximab has been described only in a few cases. Herein, we report a 25-year-old Hispanic man who presented with acute-onset SLE manifested by polyarthralgia, nephritis, seizures, pancytopenia, severe hypocomplementemia, and elevated anti-dsDNA antibodies. His disease course was complicated by DAH. His condition was refractory to high-dose intravenous (IV) methylprednisolone pulses, IV cyclophosphamide, and plasmapheresis. Given the lack of clinical response, he was started on IV rituximab 375 mg/m2 weekly for a total of four courses. He rapidly improved after the first two doses. Over the next seven months, he did not present recurrent pulmonary symptoms. Follow-up chest computed tomography did not show residual abnormalities. This case, together with other reports, suggests that rituximab is an effective therapeutic option for DAH in SLE.

Long-Term Outcomes in Puerto Ricans with Rheumatoid Arthritis (RA) Receiving Early Treatment with Disease-Modifying Anti-Rheumatic Drugs using the American College of Rheumatology Definition of Early RA.

BACKGROUND: Early treatment of rheumatoid arthritis (RA) results in better long-term outcomes. However, the optimal therapeutic window has not been clearly established. OBJECTIVE: To determine the clinical outcome of Puerto Ricans with RA receiving early treatment with conventional and/or biologic disease-modifying anti-rheumatic drugs (DMARDs) based on the American College of Rheumatology (ACR) definition of early RA. METHODS: A cross-sectional study was performed in a cohort of Puerto Ricans with RA. Demographic features, clinical manifestations, disease activity, functional status, and pharmacotherapy were determined. Early treatment was defined as the initiation of DMARDs (conventional and/or biologic) in less than 6 months from the onset of symptoms attributable to RA. Patients who received early (< 6months) and late (≥6 months) treatments were compared using bivariate and multivariate analyses. RESULTS: The cohort comprised 387 RA patients. The mean age at study visit was 56.0 years. The mean disease duration was 14.9 years and 337 (87.0%) patients were women. One hundred and twenty one (31.3%) patients received early treatment. In the multivariate analysis adjusted for age and sex, early treatment was associated with better functional status, lower probability of joint deformities, intra-articular injections and joint replacement surgeries, and lower scores in the physician's assessments of global health, functional impairment and physical damage of patients. CONCLUSION: Using the ACR definition of early RA, this group of patients treated with DMARDs within 6 months of disease had better long-term outcomes with less physical damage and functional impairment.

Olecranon Bursitis Caused by Candida parapsilosis in a Patient with Rheumatoid Arthritis.

Septic bursitis is usually caused by bacterial organisms. However, infectious bursitis caused by fungi is very rare. Herein, we present a 68-year-old woman with long-standing rheumatoid arthritis who developed pain, erythema, and swelling of the right olecranon bursa. Aspiration of the olecranon bursa showed a white blood cell count of 3.1 × 10(3)/µL (41% neutrophils, 30% lymphocytes, and 29% monocytes). Fluid culture was positive for Candida parapsilosis. She was treated with caspofungin 50 mg intravenously daily for 13 days followed by fluconazole 200 mg orally daily for one week. She responded well to this treatment but had recurrent swelling of the bursa. Bursectomy was recommended but she declined this option. This case, together with other reports, suggests that the awareness of uncommon pathogens, their presentation, and predisposing risk factors are important to establish an early diagnosis and prevent long-term complications.

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.

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.

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.