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      Sarcoidosis aguda: Variante de Síndrome de Löfgren sin eritema nodoso Translated title: Acute sarcoidosis: Erythema Nodosum-Free Variant of Löfgren’s Syndrome

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          Abstract

          El síndrome de Löfgren, es una variante aguda de la sarcoidosis, que se caracteriza por fiebre, eritema nodoso, adenomegalias hiliares pulmonares y artritis. En general, tiene un curso benigno y autolimitado, que contrasta con las formas crónicas que requieren uso de corticoides y tienen tendencia a la recidiva. Se describe aquí el caso clínico de un paciente joven, de sexo masculino, con artritis pero sin eritema nodoso, lo que dificultó el planteo diagnóstico de síndrome de Löfgren. Se realiza además una breve descripción comparativa entre la presentación clínica de la sarcoidosis crónica y el síndrome de Löfgren.

          Translated abstract

          Löfgren syndrome is an acute variant of sarcoidosis, and is defined by erythema nodosum, hilar lymphadenopathy and arthritis. Usually shows a benign self-limited course contrasting with chronic sarcoidosis with persistent relapsing presentations requiring corticosteroids most of the times. This article describes a young male patient with Löfgren syndrome with the distinct fact of the absence of erythema nodosum, which initially made sarcoidotic etiology of arthritis more difficult to think of. A brief comparative description of sarcoidosis and Löfrgen syndrome features is also made.

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          Most cited references34

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          World Trade Center "sarcoid-like" granulomatous pulmonary disease in New York City Fire Department rescue workers.

          Previous reports suggest that sarcoidosis occurs with abnormally high frequency in firefighters. We sought to determine whether exposure to World Trade Center (WTC) "dust" during the collapse and rescue/recovery effort increased the incidence of sarcoidosis or "sarcoid-like" granulomatous pulmonary disease (SLGPD). During the 5 years after the WTC disaster, enrollees in the Fire Department of New York (FDNY) WTC monitoring and treatment programs who had chest radiograph findings suggestive of sarcoidosis underwent evaluation, including the following: chest CT imaging, pulmonary function, provocative challenge, and biopsy. Annual incidence rates were compared to the 15 years before the WTC disaster. After WTC dust exposure, pathologic evidence consistent with new-onset sarcoidosis was found in 26 patients: all 26 patients had intrathoracic adenopathy, and 6 patients (23%) had extrathoracic disease. Thirteen patients were identified during the first year after WTC dust exposure (incidence rate, 86/100,000), and 13 patients were identified during the next 4 years (average annual incidence rate, 22/100,000; as compared to 15/100,000 during the 15 years before the WTC disaster). Eighteen of 26 patients (69%) had findings consistent with asthma. Eight of 21 patients (38%) agreeing to challenge testing had airway hyperreactivity (AHR), findings not seen in FDNY sarcoidosis patients before the WTC disaster. After the WTC disaster, the incidence of sarcoidosis or SLGPD was increased among FDNY rescue workers. This new information about the early onset of WTC-SLGPD and its association with asthma/AHR has important public health consequences for disease prevention, early detection, and treatment following environmental/occupational exposures.
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            Löfgren's syndrome: human leukocyte antigen strongly influences the disease course.

            Sarcoidosis may consist of a number of distinct disease entities, one of which could be Löfgren's syndrome. Patients with Löfgren's syndrome have an acute onset of erythema nodosum (EN) and/or periarticular inflammation or arthritis of the ankles, with bilateral hilar lymphadenopathy (and in some cases parenchymal infiltrates) and usually fever. There is a known association between HLA-DRB1*03 and Löfgren's syndrome. To investigate whether human leukocyte antigen type influences clinical manifestations, including the disease course in Löfgren's syndrome. We clinically characterized and HLA-DRB1 typed 301 patients with Löfgren's syndrome. A total of 275 of the patients were followed for more than 2 years and classified as having a nonresolving or a resolving disease. Almost every DRB1*03-positive patient had a resolving disease within 2 years, and 49% of the DRB1*03-negative patients developed a nonresolving disease. Mucosal granulomas were identified significantly more often in DRB1*03-negative patients. Among DRB1*03-negative patients who were treated with oral steroids at disease onset, 80% developed a nonresolving disease. Patients with Löfgren's syndrome have a different disease course depending on whether they are DRB1*03 positive or not. This observation has clinical implications, and by comparing DRB1*03-positive and DRB1*03-negative patients with Löfgren's syndrome, we can search for additional markers of importance for developing a resolving or a nonresolving disease, respectively.
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              The clinical management of sarcoidosis. A 50-year experience at the Johns Hopkins Hospital.

              Sarcoidosis is an enigmatic disease with extremely variable manifestations in pattern, severity and course. Since Longcope and Freiman's descriptive monograph in 1952 (50) summarizing the clinical findings of the first half of this century, new dimensions of assessing the disease and treatment have been added. The impact of corticosteroids is central. The present review extends the studies to the second half of this century. Earlier diagnosis is facilitated and treatment often reverses many of the disease manifestations and improves the quality and extent of life for the patient. The management issues and guidelines outlined in this paper for both intrathoracic and extrathoracic disease are based on several longitudinal studies of the sarcoidosis patients summarized here, and 50 years of clinical experience by the senior author (CJJ) at Johns Hopkins Hospital, a tertiary referral center with an active Sarcoid Clinic. Case reports are presented in the appendix. It is clear that corticosteroids are the most effective therapeutic agent for sarcoidosis, usually with impressive and prompt response. This represents the dramatic difference in this disease after 1950. No more specific or effective immunosuppressive or antiinflammatory agents have been identified. Undesirable side effects are minimal if excessive doses are avoided. The effectiveness of "steroid-sparing agents" such as methotrexate is uncertain. Although irreversible tissue damage from the disease may limit the effectiveness of treatment, benefits of corticosteroids greatly exceed the negative side effects. Since spontaneous remissions without treatment do occur, a period of observation of 2 years are more is warranted if the patient is relatively asymptomatic. Gradual radiographic progression for 2 or more years, even without major symptoms or reduction in pulmonary function, indicates the need for a trial of corticosteroid treatment, especially in white patients where symptoms may lag behind the radiographic changes. Relapses as treatment is withdrawn are frequent, especially in African-American patients, who tend to have more severe and more prolonged disease than white patients. A minimum of 1 year of treatment is recommended unless no improvement is noted after 3 months. Continued low-dose prednisone at daily doses of 10-15 mg is helpful in preventing relapses and further progression of disease. Periodic attempts at tapering are justified. Repeated relapses may indicate the need for life-long treatment. When irreversible changes are present, especially in the presence of chronic fibrotic disease, changing goals of treatment to provide optimal supportive care may represent better management than having unrealistic expectations from increased corticosteroid dosage or the addition of other potentially toxic immunosuppressive agents. Many agents related to sarcoidosis require further research. The most important question facing sarcoid researchers today is etiology. It is difficult to design specific therapy when the fundamental causes and disease mechanisms are not established. Rather than being a single disease with a single cause, it is possible that a number of genetic factors and environmental or infectious agents may result in an immune response that is manifested as sarcoidosis. Understanding basic causal mechanisms may help explain the varied disease manifestations and aid in designing curative treatments. Such etiologic questions should be explored from both a basic science and an epidemiologic approach. Therapeutic trials of new drugs such as pentoxyfylline and possibly thalidomide are needed to address their potential as well as limitations of steroid therapy. Finally, for patients who have progressed to organ failure, the problems of sarcoid recurrence in transplanted tissue, increased allograft rejection, and long-term prognosis of solid organ transplants have yet to be resolved. (ABSTRACT TRUNCATED)
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                Author and article information

                Contributors
                Role: ND
                Role: ND
                Journal
                ami
                Archivos de Medicina Interna
                Arch Med Int
                Prensa Medica Latinoamericana (Montevideo, , Uruguay )
                1688-423X
                July 2014
                : 36
                : 2
                : 79-83
                Affiliations
                [01] orgnameUdelaR orgdiv1Facultad de Medicina
                Article
                S1688-423X2014000200007
                04938513-d7d1-41a6-b658-292139cf4800

                This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

                History
                : 05 November 2013
                : 14 March 2014
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 23, Pages: 5
                Product

                SciELO Uruguay


                artritis,eritema nodoso,sarcoidosis,arthritis,erythema nodosum,Löfgren

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