Clinimetrics in Sjögrens syndrome / Clinimetría en el síndrome de Sjögren

ABSTRACT Introduction: Primary Sjögren's syndrome (pSS) is an autoimmune disease that can affect quality of life, cause disability, including progression to systemic complications in patients. In order to evaluate these components, several clinimetric scales have been used in pSS. Methods: In order to describe the most commonly used clinimetric scales in pSS, a systematic search of articles was carried out using Google Scholar, Scielo, Embase, Academic Search Ultimate, and Medline databases. Pubmed was used for the search in Medline, with the MeSH terms: 'Clinimetry'; 'Clinimetrics'; 'Quality of life'; 'Activity Index'; 'Scales'; 'Sjögren's syndrome'; linked with the Boolean connector AND. A total of 1081 articles published up to May 2018 were reduced to the 51 of the most relevant after application of inclusion criteria. Results: The most commonly used clinimetric scales in the evaluation of systemic involvement and quality of life in patients with pSS are described. Conclusion: Clinimetric methods are very useful from the point of view of follow-up, evaluation of response to treatment, perception of the disease by patients, and objective evaluation of clinical trials in pSS.

RESUMEN Introducción: El síndrome de Sjögren primario (SSp) es una enfermedad autoinmune, cuyo compromiso se puede ver reflejado en la calidad de vida, incapacidad y progresión a complicaciones en los pacientes. Con el fin de evaluar estos componentes, diversas escalas clinimétricas se han utilizado en el SSp. Métodos: Para describir las escalas de clinimetría más utilizadas en el SSp, se realizó una búsqueda de artículos en las bases de datos Google Scholar, Scielo, Embase, Academic Search Ultimate y Medline. Se empleó Pubmed para la búsqueda en Medline, con los términos MeSH: «Clinimetry¼; «Clinimetrics¼; «Quality of life¼; « Activity index¼; «Scales¼; «Sjögren's syndrome¼; enlazados con el conector booleano AND. Se incluyeron 1.081 artículos publicados hasta mayo de 2018, que luego de la aplicación de los criterios de inclusión, se redujeron a 51 con la mayor relevancia. Resultados: Se describen las escalas de clinimetría más usadas en la evaluación del compromiso sistémico y de la calidad de vida en los pacientes con SSp. Conclusión: Los métodos clinimétricos tienen gran utilidad desde el punto de vista de seguimiento, evaluación de respuesta a tratamiento, percepción de la enfermedad por parte de los pacientes y evaluación objetiva de ensayos clínicos en el SSp.

The clinical analysis of 35 patients with cutaneous sarcoidosis / 中华内科杂志

Objective To investigate clinical features of cutaneous sarcoidosis. Methods A retrospective analysis was carried out based on the clinic data of 35 patients with cutaneous sarcoidosis who were hospitalized in Peking Union Medical College Hospital during 1980-2009. They were divided into two groups, the group without systemic involvement (skin group )and the group with systemic involvement ( systemic group). Results ( 1 ) The ratio of men and women with cutaneous sarcoidosis was 1: 3. 38, and the average incident age was (47. 5 ± 10. 0) years old. The average incident age of skin group and systemic group were (41.8 ± 12.5 ) years old and ( 50. 5 ± 7. 1 ) years old, respectively. (2) The most common skin manifestation was subcutaneous nodule, followed by maculopapule and erythema nodosa. The most common involved sites were limbs. (3) The common involved systems extra-skin included the lung, joints and lymph nodes. The involvement rate of lung in cutaneous sarcoidosis of our present data was lower than those of foreign reports. However, the involvement rates of joints, lymph nodes, kidney, muscles and nervous system showed higher in our data. (4) The incidences of fatigue and weight loss in systemic group were higher than those in skin group ( P ＜ 0. 05 ). The indexes of erythrocyte sedimentation rate, C-reactive protein and rheumatoid factor in systemic group were higher than those in skin group ( P ＜ 0. 05 ). More patients in systemic group were treated with corticosteroid than that in skin group ( 95.7% vs 66.7%, P ＜ 0. 05 ).Conclusions Subcutaneous nodules are the most common and the involvement rate of lung is lower in cutaneous sarcoidosis of our present data. Compared to the patients without systemic involvement, the average incident age of systemic ones is older, the indexes of inflammation markers and the usage of corticosteroid are higher.

A Case of Adult-onset Urticaria Pigmentosa with Bone Involvement / 대한피부과학회지

Urticaria pigmentosa (UP) is the most common variant of cutaneous mastocytosis. Primarily a disease of childhood, in over one-half of the cases onset is before 2 years of age, and in 90%, the disease is confined to the skin. UP precedes the diagnosis of systemic mastocytosis and is more common in adults than in children. Therefore, systemic examination should be performed in patients with UP. We report a case of adult-onset UP with systemic involvement.

A Case of Multiple Juvenile Xanthogranuloma Involving Skin and Systemic Organs / 소아과

Juvenile xanthogranuloma(JXG) is one of the most common non-Langerhans cell histiocytosis, benign histiocytic proliferative disorder. It typically presents as a solitary, benign, rapidly growing cutaneous tumor that may regress spontaneously. Most cutaneous lesions of JXG occur in the head and neck region of infants and young children. This disease is usually present with cutaneous lesion only; visceral involvement including the lung, bone, testis, gastrointestinal tract, kidney, heart, eye and oral cavity is rare, but may affect various organs. Moreover, JXG is associated with neurofibromatosis type I and juvenile chronic myeloid leukemia. In contrast to the cutaneous form, systemic JXG may be associated with significant complications requiring aggressive medical care. The authors report a case of JXG in a 3-month-old male infant which involved systemic organs, with a brief of the literature. The patient presented with cutaneous, pulmonary, pancreatic, testicular and hepatic nodules. The cutaneous nodule biopsy shows the proliferation of histiocytes which have indistinct borders, round vesicular nuclei, foamy cytoplasm and eosinophils. The patient's follow-up was characterized by slow and progressive clinical improvement without specific treatment.

A Case of Sarcoidosis

We report a case of sarcoidosis which developed in the skin, lung and eyes in a 50-year-old woman. The skin lesions showed two types of cutaneous manifestations which were subcutaneous nodules on her back and erytrematous papules on the face for several months. Our patient's seurm angiotensin converting enzyme (ACE) level was elevated at 33.5 IU/L(reference range, 8.3-21.4 IU/L) and the purified protein derivative(PPD) skin test had a negative result. On her roentgenographic examinations, we could find multiple nodular densities with hazziness on both her lungs and radionuclides(Gallium 67) uptakes in right lower lung field. The ratio of helper to suppressor T cells was 9 in bronchial lavage. Diagnosis of sarcoidosis was made on the basis of histologic findings of non-caseating granulomas from skin and lung tissue. Treatment was initiated with oral alternate-day prednisone 40mg. The cutaneous lesions and dyspnea improved in 2 and 4 weeks respectively, and serum ACE level returned to normal in 2 months.