Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus.

OBJECTIVE: The Systemic Lupus International Collaborating Clinics (SLICC) group revised and validated the American College of Rheumatology (ACR) systemic lupus erythematosus (SLE) classification criteria in order to improve clinical relevance, meet stringent methodology requirements, and incorporate new knowledge regarding the immunology of SLE. METHODS: The classification criteria were derived from a set of 702 expert-rated patient scenarios. Recursive partitioning was used to derive an initial rule that was simplified and refined based on SLICC physician consensus. The SLICC group validated the classification criteria in a new validation sample of 690 new expert-rated patient scenarios. RESULTS: Seventeen criteria were identified. In the derivation set, the SLICC classification criteria resulted in fewer misclassifications compared with the current ACR classification criteria (49 versus 70; P = 0.0082) and had greater sensitivity (94% versus 86%; P < 0.0001) and equal specificity (92% versus 93%; P = 0.39). In the validation set, the SLICC classification criteria resulted in fewer misclassifications compared with the current ACR classification criteria (62 versus 74; P = 0.24) and had greater sensitivity (97% versus 83%; P < 0.0001) but lower specificity (84% versus 96%; P < 0.0001). CONCLUSION: The new SLICC classification criteria performed well in a large set of patient scenarios rated by experts. According to the SLICC rule for the classification of SLE, the patient must satisfy at least 4 criteria, including at least one clinical criterion and one immunologic criterion OR the patient must have biopsy-proven lupus nephritis in the presence of antinuclear antibodies or anti-double-stranded DNA antibodies.

Efficacy of mycophenolate mofetil in antimalarial-resistant cutaneous lupus erythematosus.

BACKGROUND: Mycophenolate mofetil (MMF) is an immunomodulatory drug shown to be effective in the treatment of systemic lupus erythematosus. Several anecdotal reports have suggested that MMF may be efficacious in the treatment of cutaneous lupus erythematosus (CLE). OBJECTIVES: Our objective was to summarize and report our experience with the use of MMF in patients with CLE recalcitrant to antimalarial therapy. METHODS: We retrospectively analyzed our open-label observations of 24 patients with CLE refractory to antimalarial therapy. The records of all patients visiting the Rheumatic Skin Disease Clinic at the University of Texas Southwestern Medical Center at Dallas from January 1, 2001, to July 1, 2006, were reviewed. RESULTS: MMF was tolerated well and, in conjunction with other therapies, was highly effective in the treatment of antimalarial-resistant CLE. With the addition of MMF to the existing regimen, a majority of patients achieved full control of disease signs and symptoms. All patients experienced some degree of improvement. LIMITATIONS: This is an open-label retrospective review. Severity of disease was assessed by qualitative assessment of the clinician. MMF was not used as monotherapy. CONCLUSIONS: Our results indicate that MMF, used as an additional agent in conjunction with standard therapy, is both well tolerated and efficacious in the treatment of refractory CLE. Despite the obvious limitations of the study, we believe this represents further evidence that MMF should be considered early in the treatment of patients refractory to antimalarial therapy.

Cross-sectional analysis of a collaborative Web-based database for lupus erythematosus-associated skin lesions: prospective enrollment of 114 patients.

OBJECTIVES: To assess disease severity in subsets of patients with cutaneous lupus erythematosus (CLE) by using outcome and quality-of-life measures, and to determine treatment responsiveness by establishing a Web-based database of patients with skin manifestations of lupus. DESIGN: Prospective, cross-sectional study. SETTING: University hospital cutaneous autoimmunity outpatient clinic. PATIENTS: One hundred fourteen patients who presented from January 15, 2007, to November 8, 2007, and met the criteria for having CLE or lupus-nonspecific skin disease. MAIN OUTCOME MEASURES: Scores on the CLE Disease Activity and Severity Index and the modified Skindex-29 (a quality-of-life measure) completed at each visit. RESULTS: Seven patients (6.1%) presented with acute CLE, 21 (18.4%) with subacute CLE, 77 (67.5%) with chronic CLE, 7 (6.1%) with systemic lupus erythematosus and LE-nonspecific skin lesions, and 1 (0.9%) with LE-nonspecific skin disease only. The mean baseline CLE Disease Activity and Severity Index activity/damage scores in patients with acute, subacute, and chronic CLE were 6.4/5.1, 11.1/1.6, and 7.5/10.2, respectively. The mean baseline modified Skindex-29 scores were 76.3, 79.4, and 82.7, respectively (P = .80). The disease in 11 of the patients (9.6%) was considered refractory to conventional therapies. Significantly more patients in the refractory group than the nonrefractory group were current smokers (P = .006). CONCLUSION: This Web-based database should allow collection of data related to disease activity, quality of life, and response to therapy at multiple centers.

Leflunomide as adjuvant treatment of dermatomyositis.

We report 3 cases of recalcitrant dermatomyositis that responded to the addition of daily leflunomide, a novel immunomodulatory drug that is currently used to treat rheumatoid arthritis. In our patients, leflunomide proved both safe and effective as adjuvant therapy in treating dermatomyositis.

Methotrexate in dermatology.

Methotrexate is a folic acid analog pioneered for use in inflammatory diseases by dermatologists, and used successfully for over 40 years for a wide variety of cutaneous diseases. In addition to its antiproliferative properties, methotrexate has other more recently recognized anti-inflammatory properties related to its effects on adenosine. Further research concerning its mechanism of action and genetic enzymatic variations suggest future possibilities for maximizing therapy and predicting adverse events. In this review the present authors will explore methotrexate's pharmacokinetics, mode of administration, dosing guidelines, side effect profile, and medication interactions. In addition, the present authors hope to offer practical guidelines for dose initiation and adjustment, and to summarize new research on its mechanism of action and implications for future therapy.

Tumor necrosis factor-alpha inhibitor associated ulcerative colitis.

BACKGROUND: Flares or onset of inflammatory bowel disease in association with immunosuppression has been reported in the literature. METHODS: We studied 4 cases of patients with rheumatic disease who developed or had a flare of ulcerative colitis either after initiation of or while taking a tumor necrosis factor-alpha inhibitor. RESULTS: We identified 4 patients, three male and one female. Two of the male patients had a seronegative spondyloarthropathy and one had rheumatoid arthritis. The female patient had amyopathic dermatomyositis. Two of the 4 patients had ulcerative colitis prior to tumor necrosis factor-alpha treatment. Both of these patients had quiescent ulcerative colitis that flared after they began taking etanercept. Two patients developed de novo ulcerative colitis while taking a tumor necrosis factor-alpha inhibitor. CONCLUSIONS: The data presented in these 4 cases supports a temporal relationship between initiating a tumor necrosis factor-alpha inhibitor and onset or flare of ulcerative colitis. These observations raise the possibility that tumor necrosis factor-alpha inhibitor therapy, which has been used as treatment for inflammatory bowel disease, may rarely be a factor in the development of disease.

Update on connective tissue diseases in dermatology.

Recent advances in understanding the pathogenesis of autoimmune diseases, including lupus erythematosus, dermatomyositis, and scleroderma, have allowed for reorganization of the classification of these disorders. With these novel stratifications, early identification of rheumatic skin diseases with systemic implications and consistency in designing and executing therapeutic trials will be enhanced. This review will provide a compilation of updates on epidemiology, pathology, evaluation, and classification with a predominant focus on therapeutics, reflecting the growth is this area.

Is Degos' disease a clinical and histological end point rather than a specific disease?

Degos' disease is described as a rare disorder, with approximately 100 cases detailed in the literature. Nearly all are characterized by the near "pathognomonic" appearance of porcelain-white, atrophic papules with peripheral erythema and telangiectases. Many Degos' disease variants have been described including benign cutaneous Degos' disease, familial Degos' disease, atrophie blanche with Degos'-like features, and connective tissue diseases with similar findings. The course, prognosis, and treatment have substantially varied. We present four patients: the first carries a diagnosis compatible with classic Degos' disease, the second and third demonstrate cutaneous and histological findings of Degos' disease but laboratory evidence suggestive of lupus erythematosus, while the fourth has dermatomyositis with Degos'-like lesions. Because of broad overlap in clinical and histological findings, we contend that Degos' disease may not be a specific entity, but rather, may represent a common end point to a variety of vascular insults, many of which have not been fully elucidated.

Fatal interstitial pulmonary fibrosis in anti-Jo-1-negative amyopathic dermatomyositis.

Amyopathic dermatomyositis (DM) describes a subpopulation with the cutaneous eruption of DM, but without muscle involvement. Interstitial pulmonary fibrosis is a recognized complication of DM, often correlated with antisynthetase enzymes, such as anti-Jo-1. We describe a case of fatal IPF in a patient with anti-Jo-1 antibody-negative amyopathic DM.

Persistent scarring, atrophy, and dyspigmentation in a preteen girl with neonatal lupus erythematosus.

Neonatal lupus erythematosus is an uncommon autoimmune disease with distinctive cutaneous findings. Descriptions of chronic cutaneous sequelae are rare. We describe a 12-year-old girl with persistent dyspigmentation, scarring, and atrophy as a result of neonatal lupus occurring during infancy.

Tacrolimus: farmacología y usos terapéuticos en dermatología / Tacrolimus: pharmacology and therapeutic uses in dermatology

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