Casos estandarizados en reumatología. Enfermedades autoinmunes sistémicas

Introducción. Las enfermedades autoinmunes sistémicas, denominadas también colagenosis o conectivopatías, constituyen una heterogéneo grupo de procesos que presentan en común: los fenómenos de autoinmunidad, que tienen valor patogénico y, en muchas ocasiones, diagnóstico y que posibilitan la definición de subgrupos pronósticos y terapéuticos, así como la afección simultánea de diversos órganos y sistemas corporales, circunstancia que confiere a las entidades un gran polimorfismo clínico Objetivo didáctico: facilitar el trabajo autónomo en el estudio de las enfermedades autoinmunes sistémicas, mediante el análisis de casos clínicos estandarizados que reflejan puntos clave par la toma de decisiones, tanto en el ámbito del diagnóstico como en el del tratamiento Objetivos de aprendizaje: • Asentar el concepto de enfermedad autoinmune sistémica • Reconocer la importancia de las manifestaciones clínicas en la orientación diagnóstica del paciente con enfermedad autoinmune sistémica • Reconocer la importancia de las manifestaciones analíticas en el diagnóstico de las enfermedades autoinmunes sistémicas • Sentar las bases del diagnóstico diferencial entre las diversas enfermedades autoinmunes sistémicas, • Establecer una adecuada estrategia diagnóstica y terapéutica ante una enfermedad autoinmune sistémica Habilidades transversales: • Desarrollo de la capacidad de análisis y de síntesis • Búsqueda de información • Integración de conocimientos previamente adquiridos Contexto de utilización: Se considera que los casos son apropiados como material de apoyo (trabajo autónomo) en paralelo al desarrollo de las clases magistrales y de los seminarios de enfermedades autoinmunes sistémicas en la asignatura de reumatología Por sus características (afección de múltiples órganos y sistemas corporales) los casos resultan también útiles para testar conocimiento al final de la carrera

Assessment of nutritional status by dual X-Ray absorptiometry in women with rheumatoid arthritis: a case-control study

Rheumatoid arthritis (RA) has been related to an impairment of the nutritional status. Body mass index (BMI) has been used but questions arise about how to properly evaluate nutritional status in RA patients. Few studies have evaluated it by dual-energy X-ray absorptiometry.In women with RA, to analyze:Case-control study including 89 women with RA. The control group was composed by 100 patients affected by non-inflammatory rheumatic disorders. Study variables included age, RA duration, history, activity and disability, and in relation to nutritional status: BMI, serum albumin (ALB), whole body DXA assessment, and skeletal muscle index (SMI).Mean age of patients was 62 ± 8 years, mean duration of RA was 14 ± 9 years, mean disease activity score (DAS28) was 3.7 ± 1.4 and mean Health Assessment Questionnaire was 0.88 ± 0.77. BMI was 27.43 ± 5.16 Kg/m in patients and 27.78 ± 3.98 Kg/m in controls (P: ns). ALB was within normal range in all patients.By whole body DXA, RA patients presented a statistically significant lower lean mass in all locations and lower fat mass in limbs than controls. Patients had a redistribution of fat mass to trunk. Lean mass directly correlated with fat mass.Neither BMI nor ALB correlated with DXA parameters.BMI, appendicular lean mass and SMI correlated inversely with disease duration. Trunk lean mass correlated inversely, and fat mass directly, with RA disability parameters.RA patients fulfilled criteria of sarcopenia in 44% of cases versus 19% of controls (P <.001). In RA patients, regarding SMI, BMI showed a high specificity to detect sarcopenia (94% of the patients with low BMI had sarcopenia) but low sensitivity (47% of the patients with normal BMI or overweight had sarcopenia).RA patients have an impairment of nutritional status associated to disease duration that looks like sarcopenia and that is not predicted by BMI

Sarcopenia, immune-mediated rheumatic diseases, and nutritional interventions

Introduction: Sarcopenia is defined by a loss of muscle mass and function associated with mortality, decreased physical performance, falls, and disability. Since chronic inflammation and decreased physical activity are risk factors for developing sarcopenia, it is critical to assess the role of sarcopenia in immune-mediated rheumatic diseases (IMRDs). Moreover, nutritional interventions are emerging as key modifiable and affordable options to improve physical performance in sarcopenia. Objective: The aim of this review is to critically summarize current information on the evidence linking nutritional interventions and sarcopenia in IMRDs. Methods: The search and selection of articles was performed in Medline, Dimensions.ai, Google Scholar, Cochrane Library, Epistemonikos, and Trip Database. The results were clustered into three areas: sarcopenia and IMRDs, sarcopenia and biological disease-modifying antirheumatic drugs (bDMARDs), and nutritional interventions for sarcopenia. Findings: Several cross-sectional studies have shown a higher prevalence of sarcopenia in IMRDs, such as rheumatoid arthritis. Although not fully established, evidence linking sarcopenia and other IMRDs (ankylosing spondylitis and systemic sclerosis) has been also described. For secondary sarcopenia prevention and treatment, bDMARDs' administration proved efficacy in patients with rheumatoid arthritis. Furthermore, there is growing evidence linking nutrition to the prevention and treatment of sarcopenia. Evidence linking unfavourable results in nutritional risk assessment, insufficient intake of protein, vitamin D, antioxidant nutrients, and long-chain polyunsaturated fatty acids and sarcopenia have been reported. Conclusion: Given that sarcopenia and IMRDs have strong links, further research is needed to improve patient care

Arthritis in acute febrile neutrophilic dermatosis (Sweet's Syndrome)

Sir: Acute febrile neutrophilic dermatosis or Sweet's syndrome' is an uncommon condition characterised by fever, polymorphonuclear leucocytosis, painful erythematous cutaneous plaques, and a dense dermal infiltrate of neutrophils without vasculitis at the site of the skin lesions. Although many investigators suggest that acute febrile neutrophilic dermatosis is a hypersensitivity reaction,2 no defmitive cause is known. The role of the neutrophil as a cause or effect in this syndrome has not been clarified. Acute febrile neutrophilic dermatosis is histologically and clinically mimicked by several disorders, and the differential diagnosis with pyoderma gangrenosum3 and with some reactive erythemas such as erythema multiforme2 is usually difficult

Coexistencia de dos manifestaciones osteoarticulares paraneoplásicas en un paciente afecto de adenocarcinoma pulmonar

Sr. Director: Existen descritos en la literatura una amplia variedad de trastornos musculosqueléticos paraneoplásicos. De ellos, la osteoartropatía hipertrófica (OH), la dermatomiositis y la poliartritis paraneoplásica son los que se citan con mayor frecuencia. El síndrome hombromano (SHM), variante clínica de la distrofia simpática refleja, es otra entidad reumatológica ocasionalmente asociada a neoplasia. El motivo de esta carta es comunicar el caso de un paciente afecto de adenocarcinoma pulmonar que presentó concomitantemente una OH y un SHM. Se trata de un varón de 61 años de edad, fumador de 60 paquetes/año y con criterios clínicos de bronquitis crónica, que ingresó en enero de 1988 en nuestro hospital por hemoptisis. En 1985, a raíz de ser ingresado en otro centro por un episodio de neumonía, se constató la existencia de un nódulo en LSD. Ocho meses antes de ingresar en nuestro centro el paciente inició de forma insidiosa algias en ambas regiones tibiales junto con dolor y tumefacción en rodillas. Tres meses antes del ingreso se añadió al cuadro dolor en hombro derecho junto con tumefacción difusa de la mano del mismo lado. En la semana previa al internamiento el paciente sufrió varios episodios de hemoptisis por lo que fue ingresado para estudio

Evaluation of circulating type I procollagen propeptides in patients with Paget's disease of bone

We evaluated circulating aminoterminal and carboxyterminal propeptides of type I procollagen and total alkaline phosphatase levels in eighty consecutive patients affected by Paget's disease of bone. We compared the biochemical data with the extent of bone disease calculated on the basis of the bone scintigraphic indices. Serum aminoterminal propeptide of type I procollagen levels were high in 77% of patients, serum carboxyterminal propeptide of type I procollagen levels in 22% and serum total alkaline phosphatase levels in 76%. We found significant correlations between the three markers studied. The three biochemical markers correlated significantly with the bone scintigraphic activity indices, but the highest correlation coefficient was between the aminoterminal propeptide and total alkaline phosphatase. We conclude that there is a discrepancy between serum levels of the propeptides studied in relation to Paget's disease of bone. The sensitivity of the carboxyterminal propeptide of type I procollagen in this disease is low. In contrast the aminoterminal propeptide may be as sensitive a marker for the evaluation of this disorder as total alkaline phosphatase, and in addition may be more specific

Adult-onset Still's disease with atypical cutaneous manifestations

The diagnosis of adult-onset Still's disease (AOSD) can be very difficult. There are no specific tests available, and diagnosis is usually based on a symptom complex and the well-described typical evanescent rash seen in the majority of patients. However, in recent years, other atypical cutaneous manifestations of AOSD have been reported. These atypical skin eruptions often present in addition to the typical evanescent rash but may also be the only skin manifestation, resulting in delayed diagnosis because of under-recognition. In this study, we present 3 new cases of AOSD with atypical cutaneous manifestations diagnosed during a 30-year period in our department and review 78 additional cases previously reported (PubMed 1990-2016). These 81 patients form the basis of the present analysis. The overall prevalence of atypical cutaneous manifestations in our AOSD population was 14%. These manifestations may appear at any time over the course of the disease, and usually occur in patients who have persistent and severe disease, with a considerable frequency of clinical complications (23%), including serositis, myopericarditis, lung involvement, abdominal pain, neurologic involvement, and reactive hemophagocytic syndrome. The most representative and frequent lesion among the nonclassical skin rashes is the development of persistent pruritic papules and/or plaques. Interestingly, these lesions show a distinctive histological pattern. Other, less frequently observed lesions include urticaria and urticaria-like eruptions, generalized or widespread non-pruritic persistent erythema, vesiculopustular eruptions, a widespread peau d'orange appearance of the skin, and edema of the eyelids mimicking dermatomyositis without any accompanying skin lesion. The great majority of these patients required medium or high doses of glucocorticoids (including intravenous methylprednisolone pulse therapy in some cases) and, in nearly 40%, a more potent or maintenance immunotherapy with immunosuppressant drugs and/or biologic agents (mainly anakinra or tocilizumab) to control or manage symptoms because of a polycyclic or chronic course. The development of atypical cutaneous manifestations seems to be associated with a potentially worse prognosis, with a mortality rate reaching 8% primarily because of infectious complications related to immunosuppressive therapy. In conclusion, the appearance of atypical cutaneous manifestations is not uncommon in AOSD. Recognition of this clinical variant is crucial for the early diagnosis of AOSD, as it might imply persistent disease activity and the need for more aggressive treatment

Patients' and rheumatologists' preferences for the attributes of biological agents used in the treatment of rheumatic diseases in Spain

Purpose: To define importance values assigned to attributes of biological agents (BAs) by Spanish patients with rheumatic diseases (rheumatoid arthritis, ankylosing spondylitis, and psoriatic arthritis) and rheumatologists. Patients and methods: This was an observational, cross-sectional design based upon a rank-based full-profile conjoint analysis. A literature review and four focus groups were undertaken to identify attributes and levels. An orthogonal matrix, combining the selected levels of attributes, was used to define scenarios. Participants ranked eight scenarios from 1 (most preferred) to 8 (least preferred). The relative importance (RI) of attributes was calculated. Multivariate regression analysis was performed to identify the characteristics that influenced the values of RI. A total of 488 patients (male 50.9%, mean age 50.6 [standard deviation {SD} 12.06] years, rheumatoid arthritis 33.8%, ankylosing spondylitis 32.4%, psoriatic arthritis 33.8%; mean time since diagnosis 12.6 [SD 8.2] years) and 136 rheumatologists (male 50.4%, mean age 46.4 [SD 9.1] years, mean time of practice 16.7 [SD 8.8] years) participated. Results: The ideal BAs for patients and physicians, respectively, should allow pain relief and improvement of functional capacity (RI 39% and 44.7%), with low risk of adverse events (RI 24.9% and 30.5%), a long time prior to perceiving the need for a new dose (RI 16.4% and 12.4%), and self-administration at home (RI 19.7% and 12.5%), as identified through their preferences. Conclusion: Although efficacy and safety are paramount for patients and rheumatologists to make a choice regarding BAs, the need for a low frequency of administration and the administration method also play a role as preference attributes for BAs

The value of repeat biopsy in lupus nephritis flares

Whether a repeat renal biopsy is helpful during lupus nephritis (LN) flares remains debatable. In order to analyze the clinical utility of repeat renal biopsy in this complex situation, we retrospectively reviewed our series of 54 LN patients who had one or more repeat biopsies performed only on clinical indications. Additionally, we reviewed 686 well-documented similar cases previously reported (PubMed 1990-2015). The analysis of all patients reviewed showed that histological transformations are common during a LN flare, ranging from 40% to 76% of cases. However, the prevalence of transformations and the clinical value of repeat biopsy vary when they are analyzed according to proliferative or nonproliferative lesions. The great majority of patients with class II (78% in our series and 77.5% in the literature review) progressed to a higher grade of nephritis (classes III, IV, or V), resulting in worse renal prognosis. The frequency of pathological conversion in class V is lower (33% and 43%, respectively) but equally clinically relevant, since almost all cases switched to a proliferative class. Therefore, repeat biopsy is highly advisable in patients with nonproliferative LN at baseline biopsy, because these patients have a reasonable likelihood of switch to a proliferative LN that may require more aggressive immunosuppression. In contrast, the majority of patients (82% and 73%) with proliferative classes in the reference biopsy (III, IV or mixed III/IV + V), remained into proliferative classes on repeat biopsy. Although rebiopsy in this group does not seem as necessary, it is still advisable since it will allow us to identify the 18% to 20% of patients that switch to a nonproliferative class. In addition, consistent with the reported clinical experience, repeat biopsy might also be helpful to identify selected cases with clear progression of proliferative lesions despite the initial treatment, for whom it is advisable to intensify inmunosuppression. Thus, our experience and the literature data support that repeat biopsy also brings more advantges than threats in this group. The results of the repeat biopsy led to a change in the immunosuppresive treatment in more than half of the patients on average, intensifying it in the majority of the cases, but also reducing it in 5% to 30%

A prospective study of lung disease in a cohort of early rheumatoid arthritis patients

Lung disease is common in patients with rheumatoid arthritis (RA). The onset of lung involvement in RA is not well known. The objective is to describe the features and evolution of lung involvement in early RA, its relationship with disease activity parameters, smoking and treatments. Consecutive patients with early RA without respiratory symptoms were included and tracked for 5 years. Lung assessment included clinical, radiological and pulmonary function tests at diagnosis and during follow-up. Peripheral blood parameters (erythrocyte sedimentation rate, C reactive protein, rheumatoid factor and anti-citrullinated peptide autoantibodies) and scales of articular involvement, such as DAS28-CRP, were evaluated. 40 patients were included and 32 completed the 5-year follow up. 13 patients presented lung involvement in the initial 5 years after RA diagnosis, 3 of them interstitial lung disease. Significant decrease of diffusion lung transfer capacity of carbon monoxide over time was observed in six patients, 2 of them developed interstitial lung disease. DLCO decrease was correlated with higher values of CRP and ESR at diagnosis. Methotrexate was not associated with DLCO deterioration or lung disease development. Subclinical progressive lung disease correlates with RA activity parameters. Smoking status and methotrexate were not associated with development or progression of lung disease