A review of hematopoietic stem cell transplantation for autoimmune diseases: multiple sclerosis, systemic sclerosis and Crohn's disease. Position paper of the Brazilian Society of Bone Marrow Transplantation

ABSTRACT Autoimmune diseases are an important field for the development of bone marrow transplantation, or hematopoietic stem cell transplantation. In Europe alone, almost 3000 procedures have been registered so far. The Brazilian Society for Bone Marrow Transplantation (Sociedade Brasileira de Transplantes de Medula Óssea) organized consensus meetings for the Autoimmune Diseases Group, to review the available literature on hematopoietic stem cell transplantation for autoimmune diseases, aiming to gather data that support the procedure for these patients. Three autoimmune diseases for which there are evidence-based indications for hematopoietic stem cell transplantation are multiple sclerosis, systemic sclerosis and Crohn's disease. The professional stem cell transplant societies in America, Europe and Brazil (Sociedade Brasileira de Transplantes de Medula Óssea) currently consider hematopoietic stem cell transplantation as a therapeutic modality for these three autoimmune diseases. This article reviews the evidence available.

Complete Atrioventricular Block and Cardiopulmonary Involvement in Rapidly Progressive Systemic Sclerosis

Abstract The heart and lung are target organs in systemic sclerosis (SSc) and similar symptoms (dyspnea and cough) may make the differential diagnosis between the two lesions difficult. In addition, complete atrioventricular block (CAVB) is a rare complication of this disease. This case report is about a patient with SSc and pulmonary fibrosis who was admitted to the emergency room with CAVB, heart failure (HF) and progressive worsening of the underlying disease.

Diffuse Systemic Sclerosis in a Patient with Primary Biliary Cirrhosis and Autoimmune Hepatitis Overlap Syndrome: A Case Report

A Rare Case of Overlap Syndrome with Diffuse Systemic Sclerosis, Rheumatoid Arthritis, and Cutaneous Sarcoidosis

Overlap syndrome is defined as a disease entity that satisfies the classification criteria of at least two connective tissue diseases occurring concurrently or separately in a single patient. Here, we report a rare case of a 59-year-old woman with diffuse systemic sclerosis with lung involvement-rheumatoid arthritis overlap syndrome accompanied by cutaneous sarcoidosis. Although there is no consensus for the optimal treatment of overlap syndrome to date, this case of co-existing rheumatoid arthritis and systemic sclerosis with interstitial lung disease successfully responded to abatacept.

Position article and guidelines 2018 recommendations of the Brazilian Society of Rheumatology for the indication, interpretation and performance of nailfold capillaroscopy

Abstract Nailfold capillaroscopy (NFC) is a reproducible, simple, low-cost, and safe imaging technique used for morphological analysis of nail bed capillaries. It is considered to be extremely useful for the investigation of Raynaud's phenomenon and for the early diagnosis of systemic sclerosis (SSc). The capillaroscopic pattern typically associated with SSc, scleroderma ("SD") pattern, is characterized by dilated capillaries, microhemorrhages, avascular areas and/or capillary loss, and distortion of the capillary architecture. The aim of these recommendations is to provide orientation regarding the relevance of NFC, and to establish a consensus on the indications, nomenclature, the interpretation of NFC findings and the technical equipments that should be used. These recommendations were formulated based on a systematic literature review of studies included in the database MEDLINE (PubMed) without any time restriction.

Análise funcional e anatômica anorretal de pacientes femininas portadoras de esclerodermia em um centro universitário de referência em desordens do assoalho pélvico / Functional and anatomical analysis of the anorectum of female scleroderma patients at a center for pelvic floor disorders

ABSTRACT BACKGROUND: Scleroderma or progressive systemic sclerosis is characterized by a chronic inflammatory process with proliferation of fibrous connective tissue and excessive deposition of collagen and extracellular matrix in the skin, smooth muscle, and viscera. The smooth muscle most involved in scleroderma is that of the esophagus, and dysphagia is the most commonly reported symptom. However, the internal anal sphincter may also be impaired by degeneration and fibrosis, leading to concomitant anal incontinence in scleroderma patients. These patients may neglect to complain about it, except when actively questioned. OBJECTIVE: To assess anorectal function and anatomy of female scleroderma patients with symptoms of anal incontinence through Cleveland Clinic Florida Fecal Incontinence Score (CCFIS), anorectal manometry and endoanal ultrasound at the outpatient clinic of colorectal and anal physiology, Clinics Hospital, University of São Paulo Medical School (HC-FMUSP). METHODS: Female scleroderma patients were prospectively assessed and questioned as to symptoms of anal incontinence. The anorectal manometry and endoanal ultrasound results were correlated with clinical data and symptoms. RESULTS: In total, 13 women were evaluated. Their mean age was 55.77 years (±16.14; 27-72 years) and their mean disease duration was 10.23 years (±6.23; 2-23 years). All had symptoms of fecal incontinence ranging from 1 to 15. Seven (53.8%) patients had fecal incontinence score no higher than 7; three (23.1%) between 8 and 13; and three (23.1%) 14 or higher, corresponding to mild, moderate, and severe incontinence, respectively. Ten (76.92%) patients had hypotonia of the internal anal sphincter. Three-dimensional endoanal ultrasound showed tapering associated with muscle atrophy of the internal sphincter in six cases and previous muscle defects in three cases. CONCLUSION: A functional and anatomical impairment of the sphincter is an important factor to assess in patients with progressive systemic sclerosis and it should not be underestimated.

RESUMO CONTEXTO: Esclerodermia ou esclerose sistêmica progressiva caracteriza-se por um processo inflamatório crônico com proliferação e fibrose do tecido conjuntivo e uma deposição excessiva de colágeno e matriz extracelular na pele, musculatura lisa e vísceras. A musculatura lisa mais envolvida é a esofágica e a disfagia é o sintoma mais comumente relatado. Entretanto, o esfíncter anal interno também pode ser acometido por essa degeneração e fibrose ocasionando incontinência anal nos pacientes portadores de esclerodermia. Isso pode ser omitido pelo paciente, exceto quando questionado de forma direta. OBJETIVO: Analisar a função e anatomia anorretal através do escore de incontinência anal de Cleveland Clinic Florida, manometria anorretal e ultrassom endoanal em pacientes do sexo feminino portadoras de esclerodermia e sintomas de incontinência anal atendidas no ambulatório de Fisiologia Colorretoanal no Hospital das Clínicas da Universidade de São Paulo (HC-FMUSP). RESULTADOS: Treze pacientes do sexo feminino foram avaliadas com média de idade de 55,77 anos (±16,14; 27-72 anos) e duração média da doença de 10,23 anos (±6,23; 2-23 anos). O índice de incontinência anal teve variação de 1-15, sendo que sete (53,8%) pacientes apresentavam índice inferior a 7; três (23,1%) entre 8 e 13; e três (23,1%) superior a 14, correspondendo à incontinência anal leve, moderada e grave, respectivamente. Dez (76,92%) pacientes apresentavam hipotonia do esfíncter anal interno. O estudo da ultrassonografia endoanal de três dimensões demonstrou afilamento com atrofia do esfíncter anal interno em seis casos e defeito muscular em três pacientes. CONCLUSÃO: O prejuízo funcional e anatômico do complexo esfincteriano anorretal é um importante fator a ser analisado em pacientes portadores de esclerose sistêmica progressiva e isso não pode ser subestimado.

Clinical Efficacy and Safety of Bathing with Chinese Medicine Taohong Siwu Decoction () for Treatment of Diffuse Cutaneous Systemic Sclerosis: A Randomized Placebo-Controlled Trial / 中国结合医学杂志

<p><b>OBJECTIVE</b>To examine the efficacy and safety of bathing therapy with Taohong Siwu Decoction (, TSD) in the treatment of early-stage, mild-moderate diffuse cutaneous systemic sclerosis (dcSSc).</p><p><b>METHODS</b>This randomized, placebo-controlled trial enrolled 148 men and women (18-60 years) with dcSSc (disease duration 12 months) and baseline modified Rodnan skin score (MRSS) 10. Patients were randomized into a TSD group (71 cases bathing with TSD plus oral prednisone) or control group (71 cases bathing with placebo plus oral prednisone). Bathing (40 °C, 30 min) of the upper and lower limbs was carried out once daily for 12 consecutive weeks. The primary outcome measure was MRSS; secondary outcomes were Raynaud's phenomenon (RP) score, quality of life (QOL), physician visual analogue scale (VAS), patient VAS, percent predicted diffusing capacity for carbon monoxide (DLCO), percent predicted forced vital capacity (FVC), erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) level and overall treatment effect.</p><p><b>RESULTS</b>The final analysis included 135 patients (control group, 68 cases; TSD group, 67 cases). Primary and secondary outcome measures after 2 weeks of treatment showed no improvement (versus baseline) in both groups, with no differences between groups. At 12 weeks, QOL, physician VAS, patient VAS, ESR and CRP were improved in both groups, but MRSS and RP score were improved only in the TSD group (all P<0.05). MRSS, RP score, QOL, physician VAS, patient VAS, ESR and CRP differed significantly between groups (all P<0.05). Meanwhile, the overall treatment effect was significantly higher in the TSD group than in the control group (P<0.05). Adverse events in the two groups were similar (P>0.05).</p><p><b>CONCLUSIONS</b>Bathing with TSD plus oral prednisone achieves better outcomes than oral prednisone alone in patients with dcSSc and is not associated with serious adverse events.</p>

Diffuse cutaneous systemic sclerosis: A case report

BACKGROUND: Systemic sclerosis (SSc) is a rare, connective tissue  disease  with  multisystem involvement.This is due to immunological processes,vascular endothelial cell injury  and extensive activation of fibrolast that commonly affects the skin and other internal organs such as the esophagus, lungs, heart, and kidneys. SSc has one of the highest mortality among  the  autoimmune  rheumatic  diseases,  hence  the  emphasis  on  the  early  recognition  and  management  to  prevent significant progression of the disease. CASE: A 22-year-old  female  presented  with  a  one-year history of multiple hard and hypopigmented patches on the face, neck, trunk and upper extremities. Further examination revealed mask-like facies, microstomia, frenulum sclerosis, Raynaud's phenomenon, pitted scars on the digital pulp of hands  and  sclerodactyly.Baseline  blood  chemistry,chest radiograph and electrocardiography  were  all  negative  for systemic involvement. Autoantibodies were positive for dsDNA, SS-A/Ro and Scl-70. Skin biopsy revealed sclerosing dermatitis, which was consistent with SSc. OUTCOME: The  patient  was  initially  started  with  oral prednisone 0.5 mg/kg/day and was increased to 0.75 mg/kg/day for eight weeks. Prednisone was slowly tapered to 5.0 mg/day and methotrexate 15.0 mg/week was included in  the  management for eight weeks which resulted in decreased joint pains, halted the progression of skin induration, decreased in pruritus and palmar edema. CONCLUSION: The characteristic dermatological findings of SSc are not only important signs to dermatologists, but these serves as diagnostic clues for clinicians from other disciplines as well. In our case, the presence of the autoantibody Scl-70 indicated the potential risk of pulmonary fibrosis and pulmonary arterial hypertension that accounts with high mortality.Hence,physicians should be aware of the possible risk of organ damage,even when asymptomatic because there is a high risk of disease progression. The importance of early recognition and a multidisciplinary approach lead to the good outcome in this case.

Diffuse cutaneous systemic sclerosis: A case report

@#<p style="text-align: justify;"><strong>BACKGROUND: </strong>Systemic sclerosis (SSc) is a rare, connective tissue  disease  with  multisystem involvement.This is due to immunological processes,vascular endothelial cell injury  and extensive activation of fibrolast that commonly affects the skin and other internal organs such as the esophagus, lungs, heart, and kidneys. SSc has one of the highest mortality among  the  autoimmune  rheumatic  diseases,  hence  the  emphasis  on  the  early  recognition  and  management  to  prevent significant progression of the disease. <br /><strong>CASE:</strong> A 22-year-old  female  presented  with  a  one-year history of multiple hard and hypopigmented patches on the face, neck, trunk and upper extremities. Further examination revealed mask-like facies, microstomia, frenulum sclerosis, Raynaud's phenomenon, pitted scars on the digital pulp of hands  and  sclerodactyly.Baseline  blood  chemistry,chest radiograph and electrocardiography  were  all  negative  for systemic involvement. Autoantibodies were positive for dsDNA, SS-A/Ro and Scl-70. Skin biopsy revealed sclerosing dermatitis, which was consistent with SSc. <br /><strong>OUTCOME:</strong> The  patient  was  initially  started  with  oral prednisone 0.5 mg/kg/day and was increased to 0.75 mg/kg/day for eight weeks. Prednisone was slowly tapered to 5.0 mg/day and methotrexate 15.0 mg/week was included in  the  management for eight weeks which resulted in decreased joint pains, halted the progression of skin induration, decreased in pruritus and palmar edema. <br /><strong>CONCLUSION:</strong> The characteristic dermatological findings of SSc are not only important signs to dermatologists, but these serves as diagnostic clues for clinicians from other disciplines as well. In our case, the presence of the autoantibody Scl-70 indicated the potential risk of pulmonary fibrosis and pulmonary arterial hypertension that accounts with high mortality.Hence,physicians should be aware of the possible risk of organ damage,even when asymptomatic because there is a high risk of disease progression. The importance of early recognition and a multidisciplinary approach lead to the good outcome in this case.</p>

Diffuse cutaneous systemic sclerosis: A case report

BACKGROUND: Systemic sclerosis (SSc) is a rare, connective tissue  disease  with  multisystem involvement.This is due to immunological processes,vascular endothelial cell injury  and extensive activation of fibrolast that commonly affects the skin and other internal organs such as the esophagus, lungs, heart, and kidneys. SSc has one of the highest mortality among  the  autoimmune  rheumatic  diseases,  hence  the  emphasis  on  the  early  recognition  and  management  to  prevent significant progression of the disease. CASE: A 22-year-old  female  presented  with  a  one-year history of multiple hard and hypopigmented patches on the face, neck, trunk and upper extremities. Further examination revealed mask-like facies, microstomia, frenulum sclerosis, Raynaud's phenomenon, pitted scars on the digital pulp of hands  and  sclerodactyly.Baseline  blood  chemistry,chest radiograph and electrocardiography  were  all  negative  for systemic involvement. Autoantibodies were positive for dsDNA, SS-A/Ro and Scl-70. Skin biopsy revealed sclerosing dermatitis, which was consistent with SSc. OUTCOME: The  patient  was  initially  started  with  oral prednisone 0.5 mg/kg/day and was increased to 0.75 mg/kg/day for eight weeks. Prednisone was slowly tapered to 5.0 mg/day and methotrexate 15.0 mg/week was included in  the  management for eight weeks which resulted in decreased joint pains, halted the progression of skin induration, decreased in pruritus and palmar edema. CONCLUSION: The characteristic dermatological findings of SSc are not only important signs to dermatologists, but these serves as diagnostic clues for clinicians from other disciplines as well. In our case, the presence of the autoantibody Scl-70 indicated the potential risk of pulmonary fibrosis and pulmonary arterial hypertension that accounts with high mortality.Hence,physicians should be aware of the possible risk of organ damage,even when asymptomatic because there is a high risk of disease progression. The importance of early recognition and a multidisciplinary approach lead to the good outcome in this case.

Improved Gastrointestinal Involvement in Systemic Sclerosis after Immunoglobulin Treatment / 대한내과학회지

Systemic sclerosis is an intractable clinical subset characterized by diffuse fibrosis, vasculopathy, and immune abnormalities. Diffuse cutaneous systemic sclerosis has a variety of symptoms depending on the internal organs involved, such as the lungs, heart, kidneys, and gastrointestinal system. Gastrointestinal involvement is frequent and one of the major cause of morbidity and mortality. There is no sustained, effective therapy for treating the active gastrointestinal involvement of systemic sclerosis. Although immunoglobulin is commonly used in the treatment of other autoimmune diseases, its effects in the treatment of the gastrointestinal involvement in systemic sclerosis are unclear. Here, we report a patient with a severe cough caused by uncontrolled gastrointestinal involvement in diffuse cutaneous systemic sclerosis treated with immunoglobulin.

Low vitamin D serum levels in diffuse systemic sclerosis: a correlation with worst quality of life and severe capillaroscopic findings / Baixos níveis séricos de vitamina D na esclerose sistêmica difusa: correlação com pior qualidade de vida e alterações capilaroscópicas graves

ABSTRACT Objective: The aim of this study was to analyze the correlation of vitamin D levels with clinical parameters, bone mineral density (BMD), quality of life (QoL) and nailfold capillaroscopy (NC) in patients with diffuse systemic sclerosis (SSc). Methods: Thirty-eight female patients with diffuse SSc were analyzed regarding 25-hydroxyvitamin D (25OHD) serum levels. At inclusion, organ involvement, autoantibodies, modified Rodnan skin score (mRSS), Medsger Disease Severity Index (MDSI), body mass index (BMI), BMD, NC, Short-Form-36 Questionnaire (SF-36), and Health Assessment Questionnaire (HAQ), were performed through a standardized interview, physical examination and electronic chart review. Results: Mean 25OHD serum level was 20.66 ± 8.20 ng/mL. Eleven percent of the patients had 25OHD levels ≤10 ng/mL, 50% ≤20 ng/mL and 87% ≤30 ng/mL. Vitamin D serum levels were positively correlated with BMI (r = 0.338, p = 0.038), BMD-total femur (r = 0.340, p = 0.037), BMD-femoral neck (r = 0.384, p = 0.017), SF-36-Vitality (r = 0.385, p = 0.017), SF-36-Social Function (r = 0.320, p = 0.050), SF-36-Emotional Role (r = 0.321, p = 0.049) and SF-36-Mental Health (r = 0.531, p = 0.0006) and were negatively correlated with HAQ-Reach (r = −0.328, p = 0.044) and HAQ-Grip Strength (r = −0.331, p = 0.042). A negative correlation with NC-diffuse devascularization (p = 0.029) and NC-avascular area (p = 0.033) was also observed. Conclusion: The present study provides novel evidence demonstrating that low levels of 25OHD have a negative impact in diffuse SSc QoL and further studies are needed to define whether vitamin D supplementation can improve health related QoL in these patients. The additional observation of a correlation with severe NC alterations suggests a possible role of 25OHD in the underlying SSc vascular involvement.

RESUMO Objetivo: O objetivo deste estudo foi analisar a correlação entre os níveis de vitamina D e parâmetros clínicos, densidade mineral óssea (DMO), qualidade de vida (QV) e capilaroscopia periungueal (CPU) em pacientes com esclerose sistêmica difusa (ES). Métodos: Mensuraram-se os níveis séricos de 25-hidroxivitamina D (25OHD) de 38 pacientes do sexo feminino com ES difusa. No momento da inclusão, analisaram-se o envolvimento de órgãos, autoanticorpos, escore cutâneo de Rodnan modificado (ERM), Medsger Disease Severity Index (MDSI), índice de massa corporal (IMC), DMO, CPU, Short-Form-36 Questionnaire (SF-36) e Health Assessment Questionnaire (HAQ) por meio de uma entrevista padronizada, exame físico e avaliação de prontuário eletrônico. Resultados: A média do nível sérico de 25OHD foi de 20,66 ± 8,20 ng/mL. Dos pacientes, 11% tinham níveis de 25OHD ≤ 10 ng/mL, 50% ≤ 20 ng/mL e 87% ≤ 30 ng/mL. Os níveis séricos de vitamina D estiveram positivamente correlacionados com o IMC (r = 0,338, p = 0,038), DMO-fêmur total (r = 0,340, p = 0,037), DMO-colo femoral (r = 0,384, p = 0,017), SF-36-Vitalidade (r = 0,385, p = 0,017), SF-36-Aspecto social (r = 0,320, p = 0,050), SF-36-Aspecto emocional (r = 0,321, p = 0,049) e SF-36-Saúde mental (r = 0,531, p = 0,0006) e se correlacionaram negativamente com o HAQ-Alcance (r = –0,328, p = 0,044) e HAQ-força de preensão (r = –0,331, p = 0,042). Também foi observada uma correlação negativa com a CPU- desvascularização difusa (p = 0,029) e CPU-área avascular (p = 0,033). Conclusão: O presente estudo fornece evidências novas de que níveis baixos de 25OHD têm um impacto negativo sobre a qualidade de vida de pacientes com ES difusa e que são necessários mais estudos para definir se a suplementação de vitamina D pode melhorar a qualidade de vida relacionada com a saúde desses pacientes. A observação adicional de uma correlação com alterações graves na CPU sugere um possível papel da 25OHD no envolvimento vascular subjacente da ES.

Manifestação esofágica atípica na esclerose sistêmica difusa: relato de caso / Esophageal atypical manifestation in diffuse systemic sclerosis: case report

A Esclerose Sistêmica (ES) é uma doença difusa do tecido conjuntivo caracterizada pelo envolvimento cutâneo e multissistêmico. O acometimento gastrointestinal, em especial, está presente em 90% de seus portadores e acarreta considerável prejuízo à sua qualidade de vida. A precocidade do diagnóstico do envolvimento gastrointestinal permite o estabelecimento de planos terapêuticos específicos de suas complicações. Relataremos o caso de um paciente diagnosticado com Esclerose Sistêmica do tipo difusa com manifestações pulmonares e esofagogástricas e achado atípico de acometimento esofágico.

Systemic Sclerosis is a diffuse connective tissue disease characterized by cutaneous involvement and multisystemic. The gastrointestinal involvement, in particular, is present in 90% of its carriers, and causes considerable damage to their quality of life. The early diagnosis of gastrointestinal involvement, allows for the establishment of specific treatment plans for its complications. We will report the case of a patient diagnosed with diffuse Systemic Sclerosis, presenting pulmonary and esophagogastric manifestations, as well as an atypical esophageal involvement.

Liquen esclero atrófico extragenital / Extra-Genital Atrophic Lichen Sclerosus

El Liquen esclero atrófico es una enfermedad inflamatoria crónica de la piel que afecta la epidermis y la dermis, la causa es desconocida, aunque se considera que la génesis es multifactorial. Los principales factores con los que se ha relacionado, incluyen los genéticos, inmunológicos, infecciosos, traumáticos y hormonales. Se presenta el caso de un paciente masculino de 24 años de edad con diagnóstico de Liquen esclero atrófico extra genital, con antecedentes de asma bronquial desde la infancia, hace 5 años presenta múltiples lesiones en placas acrómicas, de aspecto nacarado, algunas atróficas en su región central, asintomáticas(AU)

Atrophic Lichen Sclerosus is a chronic inflammatory disease of the skin that affects the epidermis and dermis, the cause is unknown, although the genesis is considered to be multifactorial. The main associated factors have been genetic, immunological, infectious, traumatic and hormonal. A case of a 24-year-old male patient is presented here. He has a diagnosis of extra-genital atrophic lichen sclerosus, with a history of bronchial asthma since infancy. For 5 years, this patient has multiple lesions on acrylic plaques, pearly in appearance, some atrophic in the central region, asymptomatic(AU)

Review of common conditions associated with periodontal ligament widening

PURPOSE: The aim of this article is to review a group of lesions associated with periodontal ligament (PDL) widening. MATERIALS AND METHODS: An electronic search was performed using specialized databases such as Google Scholar, PubMed, PubMed Central, Science Direct, and Scopus to find relevant studies by using keywords such as “periodontium”, “periodontal ligament”, “periodontal ligament space”, “widened periodontal ligament”, and “periodontal ligament widening”. RESULTS: Out of nearly 200 articles, about 60 were broadly relevant to the topic. Ultimately, 47 articles closely related to the topic of interest were reviewed. When the relevant data were compiled, the following 10 entities were identified: occlusal/orthodontic trauma, periodontal disease/periodontitis, pulpo-periapical lesions, osteosarcoma, chondrosarcoma, non-Hodgkin lymphoma, progressive systemic sclerosis, radiation-induced bone defect, bisphosphonate-related osteonecrosis, and osteomyelitis. CONCLUSION: Although PDL widening may be encountered by many dentists during their routine daily procedures, the clinician should consider some serious related conditions as well.

Validación del índice Duruõz en una población argentina con esclerodermia / Reliability and validity of the Duruoz Hand Index in an argentinian population with scleroderma

Objetivo: el objetivo de nuestro estudio fue adaptar y validar el Indice Duruõz (ID) en una población argentina con esclerodermia. Métodos: Para la validación, reumatólogos tradujeron al español la versión original en francés. Para evaluar la validez constructiva se utilizó la escala global visual análoga del paciente (EVA), EVA para cada una de las preguntas de la misma actividad, el cuestionario de evaluación de salud (HAQ) y el Score modificado de Rodnan para evaluar la piel. Resultados: Se incluyeron 45 pacientes con diagnóstico de esclerodermia. La correlación entre la puntuación total de ID y el EVA global del paciente fue de 0,58, con el HAQ fue 0,63 y con el Rodnan fue 0,08. El coeficiente de correlación entre el EVA y cada grupo de preguntas para la misma actividad en el ID, indicó buena correlación para las preguntas que se refieren a las actividades de cocina, así como para vestirse, de higiene, y para las preguntas de oficina. Se encontró excelente nivel de correlación con los ítems relacionados con las actividades de motricidad fina. La reproducibilidad fue de 0,88 y la confiabilidad de 0,98. Conclusión: Los resultados mostraron que el ID es un cuestionario confiable y válido para esta población argentina con esclerodermia.

Objective: the aim of our study was to adapt and to validate theDHI questionnaire in an argentinian population with scleroderma. Methods:  For validation, rheumatologists translated to Spanish the original version in French. To evaluate the construct validity, we used thepatient global visual analogue scale (VAS), VAS for questions for the sameactivity, the health assessment questionnaire (HAQ) and the Rodnan. Results: a total of 45 patients with scleroderma were includedin the study. The correlation between the total score of DHI and thepatient global VAS was 0.58, with the HAQ was 0.63 and with Rodnan0.08. The correlation coefficient between the VAS and each group of questions for the same activity in the DHI questionnaire, indicatedgood correlation for the questions that refer to activities of kitchen, aswell as for dressing, for hygiene, and for the office questions. Therewas excellent level of correlation with those related to  fine motoractivities. The reproducibility was 0.88 and the reliability was 0.98. Conclusion: The results from this study show the DHI to be a reliableand valid test for this argentinian population with scleroderma.

Surgical Treatment of Extensive Tumoral Calcinosis Associated with Systemic Sclerosis

Extensive tumoral calcinosis affecting a large joint is uncommon in patients with systemic sclerosis. We report the case of a 52-year-old female patient referred for a growing calcified mass in the shoulder. She was diagnosed with interstitial lung disease and progressive systemic sclerosis. Although the pain and disability associated with the affected joint was not severe, the patient underwent surgical excision because the mass continued to grow and was likely to produce shoulder dysfunction and skin ulceration. The patient appeared well 10 months after surgery with no signs of recurrence. This report highlights the timing and indication of surgical excision in similar cases.

Spectrum of systemic sclerosis in patients presenting to a tertiary care hospital in Pakistan

To examine the clinical and laboratory features in the patients of systemic sclerosis coming to a tertiary care hospital in islamabad, pakistan. Observational cross-sectional study. Study was conducted at the department of rheumatology in pakistan institute of medical science between May 2012 and April 2013. Both male [n=5] and female [n=40] patientsof systemic sclerosis [SSc] who presented in the rheumatology outpatient department [OPD] were recruited in the study with informed consent. All the patients enrolled in the study fulfilled the american college of rheumatology [ACR] criteria for diagnosis of SSC. The mean age of the patients was 35.77 +/- 13.10 years. Out of 45 patients, 28 [62%] had limited cutaneous systemic sclerosis[LcSS] and 17 [38%] had diffuse cutaneous systemic sclerosis [DcSS]. Mean duration of disease was found to be 4.72 +/- 4.78 years. Anti-nuclear antibody [ANA] positivity was seen in 80% of the patients. The most common clinical feature of the disease was raynaud's phenomenon [100%] followed by microstomia [98%], sclerodactyly [91%], digital pitting [89%], digital ulcers [82%] and arthralgias [73%]. Among the constitutional symptoms fatigue was the most common one reported by 93% of the patients. Clinical features of systemic sclerosis examined in this cohort of patients were found to be consistent with the findings from other asian countries

Diffuse systemic sclerosis with bullous lesions without systemic manifestations / Esclerose sistemica difusa bolhosa sem acometimentos sistemicos

Here, we describe an atypical case of systemic sclerosis in its diffuse cutaneous form with acute and rapid progression of the cutaneous condition, without any systemic manifestations and the infrequent formation of bullae, showing the importance of diagnosis and early treatment in such cases. This case also shows that special measures should be taken for bullous cutaneous lesions and ulcerations resulting from serious sclerosis, which are entry points and increase morbidity and risk of death. Other prognostic factors include age, ESR and renal and pulmonary involvement. Capillaroscopies can be useful predictors of greater severity of systemic scleroderma, revealing a greater link with systemic, rather than cutaneous, involvement.

Descrevemos um caso atípico de esclerose sistêmica em sua forma cutânea difusa com instalação aguda e rápida progressão do quadro cutâneo sem qualquer acometimento sistêmico e a infrequente formação de bolhas, demostrando a importância do diagnóstico e tratamento precoce frente a casos semelhantes e mostrando, com a experiência deste caso, que cuidados especiais devem ser tomados com as lesões cutâneas bolhosas e as ulcerações decorrentes da grave esclerose que são portas de entrada e aumentam a morbidade e risco de morte. Outros fatores prognósticos descritos são idade, VHS e envolvimento pulmonar e renal. A capilaroscopia pode ser preditor de maior gravidade da esclerodermia sistêmica, guardando maior relação com o envolvimento sistêmico do que cutâneo.

Hipertensión arterial pulmonar por ecocardiografía en pacientes con enfermedades de tejido conectivo / Pulmonary hypertension associated with connective tissue diseases

Background: Pulmonary arterial hypertension is an important cause ofcomplica-tions amongpatients with connective tissue diseases. Aim: To describe the clinical and echocardiographic characteristics ofpatients with pulmonary hypertension associated with connective tissue diseases. Material and Methods: Retrospective, observational and descriptive study. We analyzed 35 patients with pulmonary hypertension associated with connective tissue diseases. All patients were evaluated and diagnosed by at least one medical specialist in rheumatology. Pulmonary arterial hypertension was defined as a pulmonary artery systolic pressure ≥ 40 mmHg by echocardiography. The group was divided as not severe when pressures ranged from 40 to 64 mmHg and severe, when pressures were ≥ 65 mmHg. Results: The most common connective tissue disease associated with pulmonary arterial hypertension was diffuse scleroderma in 46% of cases. Eighty nine percent of patients were female. Time of evolution of the pulmonary hypertension was 18.8 ± 21.8 months. The distance walked in the six minute walk test was < 400 m both in patients with and without severe pulmonary hypertension. Fifty one percent ofpatients had pulmonary restriction. No differences in gas exchange parameters were observed between groups. Comparing echocardio-graphic findings in patients with and without severe hypertension, the former had a higher frequency ofright ventricular dilatation (85.7 and 52.3% respectively, p = 0.04), right ventricular hypertrophy (42.8 and 0% respectively, p = 0.02) and right ventricular hypokinesia (71.4 and 9.5% respectively p = < 0.01). Conclusions: Patients with severe pulmonary arterial hypertension associated to connective tissue diseases have more commonly dilated, hypertrophic and hypokinetic right ventricles.