Evaluating remission and low disease activity from the perspective of the patient with axial spondyloarthritis: The cross-sectional ConREspAx study.

OBJECTIVES: To evaluate the properties of a single question to determine patient perception of remission and disease activity; to compare this with existing definitions for remission and low disease activity (LDA). METHODS: Single-center, cross-sectional study in a tertiary care hospital. Patients with axSpA (fulfilling ASAS criteria) were consecutively included. Both the patient's perception of remission and LDA and that of the physician were evaluated with a single question. Agreement between the patients' perception and other current definitions was tested using the prevalence-adjusted and bias-adjusted kappa (PABAK). The sensitivity (S) and specificity (Sp) of current definitions of disease states were tested against the patient's perception as the gold standard. RESULTS: The study population included 105 axSpA patients (63.8% males; 67.6% with radiographic sacroiliitis). Patients considered themselves to be in remission in 21% of cases and in LDA in 51.4%; physicians considered patients to be in remission in 45.7% of cases and in LDA in 35.2%. The poorest agreement was recorded for the patient's and the physician's perception of remission. The best agreement for patients' perception of remission was for a BASDAI < 2 and normal C-reactive protein values. This definition was also the most sensitive (S=72.7%) and specific (Sp=83.1%) when the patient's perception was taken as the gold standard. CONCLUSION: In a real-life setting, the evaluation of remission by the patient through a single question was the hardest criterion to achieve, although it did prove to be a feasible, valid, and specific way to assess remission.

Rheumatoid meningitis as a complication of long-standing rheumatoid arthritis: a case treated with rituximab.

Rheumatoid meningitis is a rare complication of long-standing rheumatoid arthritis. We present the case of a 39-year-old Bolivian woman with a history of seropositive rheumatoid arthritis of 12 years of evolution without extra-articular manifestations that develops a severe headache with vomiting. The diagnosis of rheumatoid meningitis was performed based on clinical history, blood count and biochemistry results, cerebrospinal fluid analysis and cranial magnetic resonance imaging findings. High-dose intravenous glucocorticoids were started, followed by rituximab. After treatment, a significant clinical improvement was observed and repeat magnetic resonance imaging scan confirmed an improvement of the meningeal lesions.

¿Es la «fibrodisplasia osificante progresiva» una enfermedad de origen vascular? Un modelo patogénico innovador / Is «fibrodysplasia ossificans progressiva» a vascular disease? A groundbreaking pathogenic model

La fibrodisplasia osificante progresiva es la causa más grave de osificación ectópica en humanos. Se caracteriza por malformaciones esqueléticas congénitas y placas de hueso maduro (endocondral) en el músculo y en otras estructuras ricas en tejido conjuntivo. Se produce por una mutación espontánea en el gen del receptor de la activina A tipo I, similar a la activina-cinasa-2. A raíz de este hallazgo, se han producido importantes avances en el conocimiento de su base molecular y celular. Además de permitir una mejor comprensión de los mecanismos que gobiernan la osificación, evidencias recientes indican que la alteración primordial radica en mecanismos básicos de la diferenciación celular que son clave en varias vías fisiológicas y en la génesis de enfermedades de gran impacto. En el presente artículo, resumimos los últimos avances con implicaciones que trascienden los límites de esta devastadora enfermedad para postularse como un nuevo modelo dentro de la fisiopatología humana (AU)

Fibrodysplasia ossificans progressiva is the most severe and disabling disorder of ectopic ossification in humans. It is characterized by congenital skeletal abnormalities in association with extraskeletal widespread endochondral osteogenesis. Virtually all patients show the same mutation in the «activin A type-I/activin-like kinase-2» receptor encoding gene. As a result of this discovery there have been significant advances in the knowledge of the cellular and molecular basis of the disease. Besides allowing a better understanding of ossification process, recent evidence indicates that the primary disturbance lies within basic mechanisms of cell differentiation that are key in several physiological pathways and in the genesis of diseases with a major impact on health. In this article we summarize these breakthroughs, with implications that go beyond the limits of this devastating disease to insinuate a new model of human pathophysiology (AU)

Is "fibrodysplasia ossificans progressiva" a vascular disease? A groundbreaking pathogenic model.

Fibrodysplasia ossificans progressiva is the most severe and disabling disorder of ectopic ossification in humans. It is characterized by congenital skeletal abnormalities in association with extraskeletal widespread endochondral osteogenesis. Virtually all patients show the same mutation in the "Activin A type-I/activin-like kinase-2" receptor encoding gene. As a result of this discovery there have been significant advances in the knowledge of the cellular and molecular basis of the disease. Besides allowing a better understanding of ossification process, recent evidence indicates that the primary disturbance lies within basic mechanisms of cell differentiation that are key in several physiological pathways and in the genesis of diseases with a major impact on health. In this article we summarize these breakthroughs, with implications that go beyond the limits of this devastating disease to insinuate a new model of human pathophysiology.

Refractory pseudoseptic arthritis in Behçet's disease successfully treated with infliximab: a case report and literature review.

Arthritis associated with Behçet's disease is typically nonerosive and nondeforming, and most patients respond to colchicine treatment. However, destructive arthritis and refractory arthritis have also been reported on occasion. Elevated white blood cell counts may occur in synovial fluid in BD, but pseudoseptic arthritis is a very rare event in this disease. We report a patient with BD and rapidly progressive deforming pseudoseptic arthritis refractory to colchicine, corticosteroids, and methotrexate, who entered remission after infliximab treatment.

Osteoartropatía hipertrófica con acro-osteolisis y neoformación ósea en un paciente con hipertensión pulmonar primaria / Hypertrophic osteoarthropathy with acro-osteolysis in a patient with primary pulmonary hypertension

La osteoartropatía hipertrófica es una entidad caracterizada por la tríada de periostitis de huesos largos, acropaquias y artritis. Radiológicamente se distinguen 2 patrones; uno caracterizado por neoformación ósea que predomina en pacientes con patología pulmonar, y otro por acro-osteolisis que se asocia más frecuentemente con cardiopatías congénitas. Presentamos el caso de un varón de 30 años diagnosticado de hipertensión arterial pulmonar primaria desde los 2 años, que desarrolló una osteoartropatía hipertrófica con un patrón radiológico mixto (AU)

Hypertrophic osteoarthropathy is an entity characterized by a triad of periostitis of long bones, clubbing and arthritis. Radiologically there are two patterns, one characterized by new bone formation which predominates in patients with pulmonary disease, and another by acro-osteolysis that is most frequently associated with congenital heart disease. We report the case of a 30-year-old man diagnosed with primary pulmonary hypertension for two years, developing hypertrophic osteoarthropathy with a mixed radiological pattern (AU)

Hypertrophic osteoarthropathy with acro-osteolysis in a patient with primary pulmonary hypertension.

Hypertrophic osteoarthropathy is an entity characterized by a triad of periostitis of long bones, clubbing and arthritis. Radiologically there are two patterns, one characterized by new bone formation which predominates in patients with pulmonary disease, and another by acro-osteolysis that is most frequently associated with congenital heart disease. We report the case of a 30-year-old man diagnosed with primary pulmonary hypertension for two years, developing hypertrophic osteoarthropathy with a mixed radiological pattern.

Dentición anómala en niña de 8 años / Abnormal dentition in an 8 years old female child

Presentamos el caso de una paciente con acortamiento de la falange distal del primer dedo de ambas manos desde el nacimiento y con antecedentes familiares de similares anomalias. A los 8 años presentó alteraciones dentarias con hipertrofia gingival e inclusiones dentarias, y dolores lumbares y en la región femoral. Las pruebas radiológicas evidenciaron aumento de densidad ósea generalizada, hipoplasia maxilar inferior y acrosteólisis de las falanges distales. Biopsia mandibular (pieza dentaria y hueso alveolar circundante): los fragmentos óseos están constituidos por trabéculas toscas e irregulares con importante resorción y neoformación ósea. Densitometría ósea: T +5,2 en la columna lumbar y el cuello femoral. Gammagrafía ósea: captación difusa del esqueleto axial. Diagnóstico: picnodisostosis (AU)

Patient with shortening distal phalanx 1st finger both hands, from the birth and familiar precedents of similar abnormality. At the age of 8 she presented dental alterations with hipertrofia gingival and dental incorporations, and backaches and pain in femoral region. Radiology: increase of bony widespread density, hypoplasia lower jaw and acrosteolisis distal phalanxes. The jaw biopsy (dental piece and alveolar surrounding bone): bony fragments are constituted for coarse and irregular trabeculas with importantly bony resorption and newly formed. Bone densitometry: T+5.2 in c. lumbar and neck femoral. Gamma scan bone: diffuse captation of the axial skeleton. Diagnosis: picnodisostosis (AU)

Lesión quística en calcáneo. Lipoma intraóseo / Cystic lesion of the calcaneus. Intraosseous lipoma

Presentamos 2 casos de lipoma intraóseo de calcáneo, en un varón de 38 años con dolor en talón y una mujer de 27 años sin dolor en talón. La radiografías simples mostraban una lesión quística en el calcáneo, bien definida y con márgenes esclerosos. La tomografía computarizada mostró una lesión de baja densidad bien delimitada con valores de atenuación idénticos al tejido adiposo. Los hallazgos en la resonancia magnética mostraron en las imágenes T1 y T2 potenciadas una intensidad de señal similar al tejido graso subcutáneo, y en T2-STIR una señal de baja intensidad con supresión completa que indica la presencia de grasa normal. Hasta hace poco, los lipomas intraóseos sólo se podían diagnosticar con anatomía patológica, pero ahora es fácil realizar el diagnóstico radiológico con resonancia magnética (AU)

We report two cases of intraosseous lipoma in the calcaneus of a 38 year old man, complaining about heel pain and a 27 year old woman with no pain. Plain radiographs showed a well-defined cystic lesion in the calcaneus with sclerotic margins. Computed tomography (CT) detected a well-defined, low-density lesion with attenuation values equal to adipose tissue. Magnetic resonance (MR) findings show similar signal intensity with subcutaneous adipose tissue on T1-weighted and T2-weighted images, and STIR-T2 imaging showing low signal intensity with complete suppression indicating the presence of normal fat. As a result, at first intraosseous lipomas could only be identified pathologically, but now it is easy to perform radiological diagnosis using MR (AU)

[Cystic lesion of the calcaneus. Intraosseous lipoma]. / Lesión quística en calcáneo. Lipoma intraóseo.

We report two cases of intraosseous lipoma in the calcaneus of a 38 year old man, complaining about heel pain and a 27 year old woman with no pain. Plain radiographs showed a well-defined cystic lesion in the calcaneus with sclerotic margins. Computed tomography (CT) detected a well-defined, low-density lesion with attenuation values equal to adipose tissue. Magnetic resonance (MR) findings show similar signal intensity with subcutaneous adipose tissue on T1-weighted and T2-weighted images, and STIR-T2 imaging showing low signal intensity with complete suppression indicating the presence of normal fat. As a result, at first intraosseous lipomas could only be identified pathologically, but now it is easy to perform radiological diagnosis using MR.