Anti-interleukin-6 receptor antibody for the treatment of remitting seronegative symmetrical synovitis with pitting oedema: a new outlook?

We present the case of an elderly man with a small-joint polyarthritis, accompanied by pitting oedema, involving hands and feet, raising clinical suspicion of remitting seronegative symmetrical synovitis with pitting oedema (RS3PE). Treatment with corticosteroids was initiated with significant improvement, but unacceptable iatrogeny ensued, and tapering was not possible without disease flare-up. A trial of tocilizumab allowed disease activity control, slow weaning of corticosteroids and, ultimately, its suspension. RS3PE is a rare rheumatological entity, initially thought to be a variant of rheumatoid arthritis (RA), with shared traits with polymyalgia rheumatica (PMR), and other seronegative spondyloarthropathies, thereby implying a shared pathophysiological background. Elevated levels of interleukin 6 (IL-6) are found in patients with RA, have shown to mirror disease activity in PMR and have also been described in the serum and synovial fluid of patients with RS3PE. Tocilizumab, an anti-IL-6 receptor antibody, shows auspicious results in several other rare rheumatic diseases other than RA.

Progesterone hypersensitivity: a cyclical history.

Hypersensitivity reaction to progesterone is rare, with less than 200 cases reported. It occurs mainly in women in their third decade of life and can have a heterogeneous presentation (cutaneous and/or systemic), with temporal relation to serum levels of progesterone. Diagnosis is based on history and physical examination, supported by skin tests. We describe the case of a woman in her late 20s with pruritic erythema on the chest that manifested in the luteal phase of the menstrual cycle, with spontaneous resolution in less than 5 days, without secondary scarring. The histological diagnosis was non-specific, with evidence of interface dermatitis, but positive skin tests supported the diagnosis of autoimmune progesterone dermatitis. It is intended to alert to the manifestations of a pathology that is rare and difficult to diagnose but with a great impact on daily life.

A Case of Recurrent Idiopathic Pyoderma Gangrenosum.

Pyoderma gangrenosum (PG) is a rare chronic neutrophilic dermatosis that can be associated with underlying conditions, such as inflammatory bowel disease and neoplasms, or can be idiopathic. Classically, it presents as painful skin lesions. We present a case of a 54-year-old woman who got a synovial cyst removed from her left hand, which later aggravated into a non-healing wound, and subsequently a painful necrotic ulcer. The histological pattern combined with the clinical features suggested PG. General wound care was performed, associated with topical tacrolimus and oral corticotherapy with a good response. Three similar episodes with lesions scattered over the body followed and required a combination of other pharmacological alternatives. An extensive etiological study was carried out to screen secondary causes without any relevant findings. Therefore, an idiopathic relapsing PG was assumed. PG is poorly understood, underdiagnosed and hard to treat. It has a clear impact on the quality of life of the patient, so high suspicion and timely treatment are essential to minimize complications.

Plaque morphea with neurological involvement­an extraordinary uncommon presentation.

Localized scleroderma is traditionally considered to be limited to the skin, subcutaneous tissue, underlying bone, and in the craniofacial subtype, also nervous system involvement. However, recent studies have also described other systemic manifestations in these patients. Despite many reports of neurological involvement in patients with the craniofacial linear localized scleroderma, it is extremely rare in patients with the other subtypes of localized scleroderma. Here, we report an extraordinary case of localized scleroderma en plaque (classic morphea), located to the upper trunk and neck, associated with neurological manifestations presented as seizures. Magnetic resonance imaging of the brain showed focal lesions on the contralateral side to the skin involvement. This case is extremely relevant not only due to its rarity, but also because it supports the idea that the pathogenesis of the localized scleroderma is related to a systemic autoimmune process.

14th International Congress on Antiphospholipid Antibodies Task Force Report on Catastrophic Antiphospholipid Syndrome.

The 'Task Force on Catastrophic Antiphospholipid Syndrome (CAPS)' was developed on the occasion of the 14th International Congress on Antiphospholipid Antibodies. The objectives of this Task Force were to assess the current knowledge on pathogenesis, clinical and laboratory features, diagnosis and classification, precipitating factors and treatment of this condition in order to address recommendations for future research. This article summarizes the studies analyzed by the Task Force, its recommendations and the future research agenda.

The hyperferritinemic syndrome: macrophage activation syndrome, Still's disease, septic shock and catastrophic antiphospholipid syndrome.

BACKGROUND: Over the last few years, accumulating data have implicated a role for ferritin as a signaling molecule and direct mediator of the immune system. Hyperferritinemia is associated with a multitude of clinical conditions and with worse prognosis in critically ill patients. DISCUSSION: There are four uncommon medical conditions characterized by high levels of ferritin, namely the macrophage activation syndrome (MAS), adult onset Still's disease (AOSD), catastrophic antiphospholipid syndrome (cAPS) and septic shock, that share a similar clinical and laboratory features, and also respond to similar treatments, suggesting a common pathogenic mechanism. Ferritin is known to be a pro-inflammatory mediator inducing expression of pro-inflammatory molecules, yet it has opposing actions as a pro-inflammatory and as an immunosuppressant. We propose that the exceptionally high ferritin levels observed in these uncommon clinical conditions are not just the product of the inflammation but rather may contribute to the development of a cytokine storm. SUMMARY: Here we review and compare four clinical conditions and the role of ferritin as an immunomodulator. We would like to propose including these four conditions under a common syndrome entity termed "Hyperferritinemic Syndrome".

Long-term complications of past glucocorticoid use.

Glucocorticoids (GC) are essential in the management of several medical conditions but its long-term use is associated with complications in diverse organs and systems. The aim of the present study is to review the long-term complications of past GC use. Permanent damage related to GC can affect patient's life even years after its withdrawal. Classical examples are cataracts and esthetic problems like skin atrophy, striae, acne and obesity. Interestingly, for some complications, the risk of an incident event can persist for past GC use. Higher risks of osteoporosis, osteonecrosis, cardiovascular disease, infections and cancer have been associated with prior GC therapy. These evidences reinforce the importance of limiting our GC prescriptions at its lower possible dose.

Meta-análisis de estudios realizados en la Facultad de Medicina de la Universidad Nacional de San Agustín de Arequipa sobre apendicitis aguda

OBJETO DE ESTUDIO: Clasificar las investigaciones(objetivos) de acuerdo a la historia natural de enfermedad y determinar la validez de la información dada, mediante la evaluación de sus conclusiones provengan o no de objetivos planteados a priori, con respecto a apendicitis aguda. DISEÑO: El presente trabajo es un estudio Transversal de diagnóticoO y estadío. UBICACION: Facultad de Medicina, Universidad Nacional de San Agustín, Arequipa. SUJETO DE ESTUDIO: Constituído por los 71 objetivos y 348 conclusiones de las 25 tesis motivo del estudio,tomadas de los listados oficiales de la Biblioteca de Biomédicas de la UNSA. METODOS: Se realizó un análisis, haciendo uso de una nueva técnica, (el meta-análisis), mediante el cual se determinó la validez de las concluciones emitidas, provengan o no de objetivos planteados a priori, en el primer caso a través del análisis de los objetivos, se determinó si estos provenían de un problema planteado, pudieron ser verificados y justificados, y finalmente si utilizaron las pruebas estadísticas correspondientes; en el segundo caso el análisis efectuado fue similar, con la diferencia que en este caso se partió de las concluciones que no provenían de objetivos planteados a priori. RESULTADOS: Se encontró que de las 25 tesis, un 60.55 por ciento fueron temas relacionados con frecuencia de enfermedady diagnóstico. De las concluciones que provenían de objetivos planteados a priori sólo 1.19 por ciento fueron consideradas como válidas desde el punto de vista científico, y de las que provinieron de objetivos planteados a priori fueron sólo 37.8 por ciento. CONCLUSIONES: Según la historia natural de la enfermedad se encontró que la mayoría de los estudios realizados abarcan aspectos relacionados con la frecuencia de enfermedad y diagnóstico. Se encontró una conclusión valida desde el punto de vista científico que provenía de un sólo objetivo planteado a priori. De las conclusiones que no provenían de objetivo, que conformaban la mayoría fueron consideradas válidas menos del 50 por ciento