ABSTRACT
Resumen La enfermedad de Still del adulto (ESA) es una enfermedad reumática e inflamatoria, infrecuente, de etiología desconocida. Su presentación clínica es variada y sus síntomas más frecuentes son fiebre, artralgias, exantema asalmonado evanescente, odinofagia, adenopatías, hepatoesplenomegalia, serositis y presencia en laboratorio de leucocitosis (neutrofilia), hiperferritinemia asociado a ausencia de anticuerpos. El diagnóstico es clínico y por exclusión. Existen criterios clasificatorios como son los de Yamaguchi1, Cush y Fautrel2,3 que ayudan a la orientación diagnóstica. El tratamiento se define según la presentación y la evolución clínica1. Se describen las características clínicas, diagnósticas, tratamiento y evolución de siete pacientes con ESA.
Abstract Adult Still's disease (ASD) is an uncommon rheumatic and inflammatory disorder of unknown etiology. The major clinical features include fever, arthralgia, transient salmon-pink rash, odynophagia, lymphadenopathy, hepatosplenomegaly, serositis, and laboratory findings such as leukocytosis with neutrophilia, hyperferritinemia, and negative immunologic laboratory testing. ASD diagnosis is reached by exclusion. Different classification criteria such as Yamaguchi, Cush and Fautrel have been developed for the identification of ASD. Treatment is based on the degree of disease activity and clinical response1. We describe clinical manifestations, diagnosis, treatment, and prognosis of seven patients with adult's Still disease.
Subject(s)
Still's Disease, Adult-Onset , Rheumatic Diseases , FeverABSTRACT
Adult Still’s disease is rare and may present as pyrexia of unknown origin. Due to lack of expertise, diagnosis may be delayed inadvertently. The patient usually presents with spiked fever, polyarthralgia or arthritis, evanescent skin rash, non-purulent pharyngitis, lymphadenopathy and hepatosplenomegaly. Leukocytosis, predominantly of neutrophils, elevated erythrocyte sedimentation rate and C-reactive protein without obvious infection are the hallmarks of the disease. Delay in diagnosis may expose the patient to the side effects of antibiotics as they are repeatedly prescribed in view of elevated leukocytes. The majority of patients report pain in the throat without evidence of infection. This was an important clue to our diagnosis of this patient. Grossly elevated serum ferritin is diagnostic of adult onset still's disease. As the white cell counts are grossly elevated, a bone marrow examination to rule out hematological malignancy may be mandatory. Serum ferritin value has prognostic value too. Minor illness may respond to non-steroidal anti-inflammatory drugs (NSAIDs), but steroids are the mainstay of the treatment. Methotrexate is of additional value for those presenting predominantly with arthritis. Anakinra, Infliximab and Tocilizumab are other options. Those patients presenting with severe disease and organ involvement require high dose intravenous steroids followed by high dose oral steroids.
ABSTRACT
La enfermedad de Still del adulto (ESA) es un proceso inflamatorio sistémico, de etiología desconocida, que se caracteriza por fiebre, artritis y eritema evanescente, además de valores elevados de ferritina sérica. Sin embargo, hasta la fecha, no hay una prueba definitiva de laboratorio o de imagen disponible para su diagnóstico, por lo tanto la ESA es un diagnóstico de exclusión. Presentamos el caso de una mujer de 44 años con manifestación cutánea atípica de ESA y cuadro clínico de 1 año de evolución caracterizado por fiebre de 40°C, linfadenopatía, hiperferritinemia, y que en la sistemática de estudio presentó positividad para anti-CCP (anticuerpo antipéptido cíclico citrulinado).
Adult Still's disease (ASD) is a systemic inflammatory process, of unknown etiology, characterized by fever, arthritis and evanescent erythema, in addition to elevated serum ferritin values. However, to date, there is no definitive laboratory or imaging test available to diagnose it, therefore ASD is a diagnosis of exclusion. We present the case of a 44-year-old woman with atypical cutaneous manifestation of ESA and positivity of ACPA (anti-cyclic citrullinated peptide antibody).
Subject(s)
Still's Disease, Adult-Onset , Ferritins , Anti-Citrullinated Protein AntibodiesABSTRACT
Objetivo: Describir las características clínicas, paraclínicas y manejo de la enfermedad de Still del adulto (E.S.A.). Métodos: Análisis retrospectivo de una cohorte de 24 pacientes. Resultados: Las características clínicas fueron: fiebre (100%), rash (79.1%) y artritis (66.66%). Leucocitosis (75%) fue la alteración paraclínica más frecuente, seguida por un incremento de la PCR, VSG y ferritina en 70.8%, 66.65% y 62.5%, respectivamente. El 50% de los pacientes presentaron un curso monocíclico; el 33%, policíclico sistémico y el 17%, policíclico articular. No se presentaron casos de monocíclico poliarticular. Se evidenciaron manifestaciones inusuales de enfermedad tales como: urticaria en 7 pacientes (29%); enfermedad pulmonar intersticial, en 6 pacientes (25%); meningitis aséptica, 4 pacientes (16.6%) y SDRA, en 4 pacientes (16.6%). Por su parte, todos los pacientes recibieron esteroides y 9 de ellos (37.5%) necesitaron tratamiento con pulsos de metilprednisolona. Dentro de las drogas modificadoras, el metotrexate fue el medicamento más usado en 15 casos (62.5%), seguido por cloroquina sola o combinada en 11 pacientes (45.8%). Además, 3 pacientes (12.5%), requirieron tratamiento con terapia anti TNF por pobre respuesta a la terapia convencional. Fallecieron 2 pacientes (8.4%) asociados a cuadros de SDRA. Conclusiones: La ESA tiene un amplio y difícil diagnóstico diferencial a pesar de manifestaciones clásicas. La presencia de expresiones clínicas poco usuales puede retardar el diagnóstico y tratamiento de ESA. La ESA no es una enfermedad benigna y puede cursar con alta mortalidad cuando se asocia a manifestaciones pulmonares severas.
Purpose: describe the clinical and laboratory finding, as well as the treatment, of adult still's disease (ASD). Methods: retrospective analysis of a 24 patients cohort. Results: clinical features were: fever (100%), rash (79.1%) and arthritis (66.6%). Most frequent laboratory alterations were: leukocytosis (75%), followed by increased PCR, VSG and ferritin, 70.8%, 66.65% and 62.5% respectively. 50% of the patients showed a monocyclic course, while 33% showed polycyclic systemic and 17% joint polycyclic. No joint monocyclic cases were found. Besides, some unusual manifestations such urticaria in 7 patients (29%); interstitial lung disease in 6 patients (25%); aseptic meningitis in 4 patients (16.6%) and SDRA in 4 patients (16.6%) were found. All the patients received steroid and 9 of them (37.5%) required therapy with IV methilprednisolone. Methotrexate was the most used medication between all; DMARD in 15 cases (62.5%), followed by chloroquine, alone or in combination, in 11 patients (45.8%). Besides, 3 patients (12.5%) required treatment with anti TNF due to poor response to conventional therapy. 2 patients died (8.4%) due to adult respiratory distress syndrome. Conclusions: ASD has a broad and difficult differential diagnosis despite classical features. The presence of unusual manifestations may delay the diagnosis and treatment of this disease. ASD in not a benign disease and may appear with high mortality when it is associated with severe lung involvement.
Subject(s)
Humans , Cohort Studies , Still's Disease, Adult-Onset , Signs and Symptoms , Patient Care Management , Disease Management , DiagnosisABSTRACT
Objective: This article is an attempt to review recent literature regarding pathogenesis and clinical and laboratory findings in adult-onset Still's disease (AOSD). Materials and methods: A search was conducted in PubMed and Ovid for English language publications, using individual or linked search terms "adult-onset Still's disease," "adult Still's disease," "Still's disease," "AOSD," and other related terms, from 1996 to 2009, and the clinically relevant articles were subsequently selected. Results: More than 1000 titles were reviewed by the authors, and the most important concepts were selected from 143 full-text articles. Conclusion: Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology and pathogenesis, usually presenting with high spiking fever accompanied by systemic manifestations. The disease is an entity with heterogeneous pathology; and diverse suggested etiologies, clinical manifestations and prognoses. There is no single diagnostic test for AOSD; rather, the diagnosis is based on a set of criteria, the most important of which are indeed clinical, but they also include paraclinical ones. Treatment aims at both minimizing inflammation and halting disease progression. For the former, nonsteroidal anti-inflammatory drugs have limited efficacy; so glucocorticoids in conjunction with disease-modifying antirheumatic drugs are also used. Novel therapeutic approaches such as anti-tumor necrosis factor blockade and monoclonal antibodies are promising.
Subject(s)
Adult , Humans , Still's Disease, Adult-Onset/diagnosis , Still's Disease, Adult-Onset/physiopathology , Still's Disease, Adult-Onset/therapyABSTRACT
Objective:To study the changes of serum IL 1?,IL 6,IL 8,TNF ? and sIL 2R and its relationship with disease activity in adult Still disease patients.Methods:Serum levels of IL 1?,IL 6,IL 8,TNF ? and sIL 2R were assessed by ELISA in 18 adult Still disease patients before and one month after treatment with prednisone.Results: Active adult Still disease patients had significant elevated erythrocyte sedimentation rate (ESR) and serum levels of C reactive protein (CRP),IL 1?,IL 6,IL 8,TNF ? and sIL 2R.After one month treatment with prednisone,ESR and serum levels of CRP,IL 6,TNF ? and sIL 2R significantly decreased.There was a significant correlation between ESR values and serum IL 6,TNF ? and sIL 2R levels,and between serum CRP levels and IL 6,TNF ? and sIL 2R levels.Serum IgG,IgA and IgM levels in active adult Still disease patients were similar to those in healthy controls.Conclusion: There are no changes in serum immunoglobulins but significant increases of serum IL 1?,IL 6,IL 8,TNF ? and sIL 2R levels in active adult Still disease.Serum levels of IL 6,TNF ? and sIL 2R are related to the activity of adult Still disease.