To describe and compare the diagnosis, demographics and management of systemic lupus erythematosus (SLE) related versus idiopathic acute transverse myelitis during the initial presentation of the disease. We undertook a chart review of the hospital records of patients admitted to our hospital from 1994 until 2007 and had the diagnosis of SLE related and idiopathic acute transverse myelitis. Demographics, laboratory and imaging studies, diagnosis and treatment were recorded in both groups and analyzed in a case control fashion. We identified 15 patients with SLE-related acute transverse myelitis (SLE-ATM) and 39 idiopathic (I-ATM) cases between 1994 and 2007. Patients with SLE were more likely to be African American, have CNS demyelinating lesions on MRI, a high IgG% on their CSF analysis and a higher sedimentation rate on presentation. Treatment with high-dose steroids was instituted in both groups of patients, though SLE patients had a longer hospital stay by an average of 5 days. SLE-ATM patients were more likely to be African American as compared to I-ATM patients, have CNS demyelinating lesions on MRI, a high IgG% on CSF analysis and a higher sedimentation rate on presentation. The hospital stay for SLE patients was 5 days longer than the idiopathic patients. This study underlines the importance of early diagnosis of patients who develop ATM related to SLE.
Lung herniation is a relatively rare clinical entity that is most commonly either congenital or acquired traumatically. We describe a case of spontaneous lung herniation secondary to acute cough in an obese male smoker complicated by contralateral acute lung injury and systemic inflammatory response syndrome (SIRS). Mechanisms of lung herniation, classification, diagnosis, and management will be discussed.
Objectives To compare the therapeutic effectiveness of corticosteroids (CS) alone versus CS plus D-penicillamine (D-Pen) in severe eosinophilic fasciitis. Methods Long term prospective non-randomized trial of D-Pen plus CS vs. CS alone in patients with severe eosinophilic fasciitis, defined as clinically apparent cutaneous fibrotic involvement affecting greater than 15% body surface area (BSA), or greater than 10% BSA with joint flexion contractures. Results Sixteen patients with severe eosinophilic fasciitis entered the study. Ten patients received D-Pen plus CS and 6 CS alone. Affected BSA decreased from an average of 29% to 8.9% in the D-Pen plus CS group compared to a decrease in affected BSA from 28% to 22.83% in the CS alone group. The reduction in affected BSA in the D-Pen plus CS group was significantly greater than in the CS alone group (p= 0.038). Clinical improvement occurred in all D-Pen plus CS patients, compared to only 33.3% of CS alone patients (p=0.008). There was no difference in overall adverse events frequency between the groups (p=0.60). The most common adverse event in the D-Pen plus CS group was proteinuria (33.3%). However, proteinuria also occurred in 16.6% in the CS-alone group. Conclusions Treatment with CS alone failed to induce clinical improvement in the majority of the severe eosinophilic fasciitis patients. In contrast, D-Pen plus CS resulted in significantly greater clinical improvement. These results suggest that initial treatment of severe eosinophilic fasciitis with CS alone is not sufficient for optimal therapeutic response and that addition of an antifibrotic agent results in an improved outcome.
IntroductionVitamin D has become an area of intensive scrutiny, both in medical and lay literature. However, there are limited data to suggest proper repletion regimens for those patients who have hypovitaminosis D. Consequently, various methods are used in clinical practice. The aim of this study was to assess the efficacy of various treatment strategies for hypovitaminosis D in an ambulatory internal medicine practice.MethodsA retrospective chart review between October 2005 and June 2010 of a suburban internal medicine practice was performed via query of the electronic medical record (Centricity, General Electric Healthcare, UK). Patients with a 25-hydroxyvitamin D concentration less than 32 mg/dl were identified and treated. Treatment success was defined as 25-hydroxyvitamin D concentrations greater than 32 mg/dl. Statistical analysis to assess changes in vitamin D level controlling for season, comorbidities, and demographics were used.ResultsA total of 607 treatment episodes were identified, with 395 excluded due to lack of follow-up vitamin D level within 16 weeks, no treatment documented, topical treatment, doxercalciferol treatment, or non-compliance. Of the remaining patients, there were 212 treatment instances on 178 patients. Ergocalciferol 50,000 international units (IU) was used most frequently (71.4% of the time.). A higher initial vitamin D level was positively associated with treatment success (adjusted odds ratio = 1.11, p=0.002). Increased doses of ergocalciferol increased the likelihood of treatment success (p=0.0011). Seasonal variation was related to posttreatment 25-hydroxyvitamin D concentration as was body mass index (BMI) (p=0.003 and p=0.044).ConclusionPretreatment levels of 25-hydroxyvitamin D, BMI, season, and vitamin D dose are predictors of successful hypovitaminosis D treatment. Our data suggest that patients with initial 25-hydroxyvitamin D concentrations of <20 should be treated with a higher total dose of ergocalciferol than 50,000 IU for 8 weeks. Further studies, including prospective, randomized trials, are needed to determine an optimal treatment protocol to account for the numerous variables.
Background Eosinophilic fasciitis is an uncommon cutaneous fibrosing disorder characterized by fascial fibrosis often accompanied by tissue eosinophilic inflammation and peripheral eosinophilia. Corticosteroids (CS) are used as a first line treatment. Despite of its high relapse rate, there is no consensus on the use and choice of additional treatment. Objectives To evaluate the efectiveness of D-Penicillamine (D-Pen) plus CS versus CS alone for the treatment of severe eosinophilic fasciitis. Methods A 20 year (1993-2013) retrospective chart review identified 16 patients with clinical and histopathological diagnosis of severe eosinophilic fasciitis. This was defined as clinically apparent fibrotic involvement of more than 15% of the body suface area and/or the presence of fibrotic lesions crossing joints in patients with confirmed histopathology who had been treated with either D-Pen plus CS or CS alone. Analysis of disease characteristics, comorbidities, efficacy, adverse events (AE) and relapse was performed in this cohort. Results Eosinophilic fasciitis lesions involved an average of 25% of BSA and 68.8% of the subjects developed a joint contractures during the course of the disease. 10/16 (62.5%) patients were treated with D-Pen plus CS and 6/16 (37.5%) with CS alone. Both groups were comparable in terms of involved body surface area, time from the first symptoms, age and co-morbidites. All of the patients initially treated with D-Pen plus CS, responded to the treatment. In contrast, only 33.3% of patients treated with CS alone had a favorable response (p=0.008). Rates of AEs were not statistically significant among the groups (80% vs 66.67% in the group treated with D-Pen plus CS vs CS alone). The most common AE reported in the D-Pen plus CS group was proteinuria which occurred in 33.3% of the subjects. However 16.6% of the patients on CS alone also developed proteinuria. Figure 1. Clinical outcomes of patients treated with CS alone and D-Pen plus CS. Conclusions In this cohort with severe eosinophilic fasciitis, a very high rate of subsequent joint contractions was observed. The treatment with D-Pen plus CS was associated with a significantly greater improvement as compared to CS alone. Proteinuria was unexpectedly common in both groups although it was more frequent in the D-Pen plus CS group. A high frequency of relapse was seen in both treatment groups. References Shulman LE. Diffuse Fasciitis with Hypergammaglobulinemia and eosinophilia: A new syndrome. J Rheumatol 1974; (suppl 1) 1:46 Barnes L, Rodnan GP, Medsger TA, Short D. Eosinophilic fasciitis. A pathologic study of twenty cases. Am J Pathol. 1979 Aug;96(2):493-518. Lebeaux D, Sène D. Eosinophilic fasciitis (Shulman disease). Best Pract Res Clin Rheumatol. 2012 Aug;26(4):449-58 Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.4513
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