A comparative study on clinical and serological characteristics between patients with rhupus and those with systemic lupus erythematosus and rheumatoid arthritis.

BACKGROUND: The concomitant presence of two autoimmune diseases - systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA) - in the same patient is known as rhupus. We evaluated a group of patients with rhupus to clarify further their clinical, serological and immunogenic features in a multi-centre cohort. In addition, the study aimed to explore the utility of the 2019 European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) SLE classification criteria in our group of patients with rhupus. METHODS: This was a cross-sectional study. We included rhupus patients from 11 different rheumatology departments, and compared them to SLE and RA patients at a ratio of 2:1. All information was recorded following a pre-established protocol. RESULTS: A total of 200 patients were included: 40 rhupus patients and 80 each of SLE and RA patients as controls. Disease duration was similar among SLE and rhupus groups (around 13 years), but the RA group had a significantly lower disease duration. Main clinical manifestations were articular (94.2%), cutaneous (77.5%) and haematological (72.5%). Rhupus patients had articular manifestations similar to those expected in RA. Only 10% of rhupus patients had renal involvement compared with 25% of those with SLE (p < 0.05), while interstitial lung disease was more common in patients affected by RA. The 2019 EULAR/ACR SLE criteria were met in 92.5% of the rhupus patients and in 96.3% of the SLE cohort (p > 0.05). Excluding the joint domain, there were no differences between the numbers of patients who met the classification criteria. CONCLUSION: Rhupus patients follow a particular clinical course, with full expression of both SLE and RA in terms of organ involvement, except for a lower prevalence of kidney affection. The new 2019 EULAR/ACR SLE criteria are not useful for differentiating SLE and rhupus patients. A new way of classifying autoimmune diseases is needed to identify overlapping clusters.

Dual targeted therapy in patients with psoriatic arthritis and spondyloarthritis: a real-world multicenter experience from Spain.

Dual targeted therapy (DTT) has emerged as a promising approach in patients with refractory spondyloarthritis (SpA) or psoriatic arthritis (PsA) and extra-musculoskeletal manifestations of both diseases, but its effectiveness/safety ratio still remains unclear. This is a retrospective, real-world multicenter study in refractory SpA and PsA patients with simultaneous use of two biological or synthetic targeted agents. Effectiveness was assessed using Ankylosing Spondylitis Disease Activity Score with C-reactive protein (ASDAS-CRP) and Disease Activity in Psoriatic Arthritis (DAPSA) Score. We identified 39 different DTT combinations in 36 patients (22 SpA; 14 PsA), 25 of them with concomitant inflammatory bowel disease. The most commonly used combinations were TNF inhibitor plus antagonist of the IL12/23 pathway, followed by TNF inhibitor plus IL-17 antagonist. During a median exposure of 14.86 months (IQR 8-20.2), DTT retention rate was 69.4% (n=25/36; 19 SpA, 6 PsA). Major clinical improvement (change in ASDAS-CRP > 2 or improvement > 85% in DAPSA) was achieved in 69.4% of patients (n=25/36 therapeutical combinations; 17/21 SpA, 8/15 PsA), with a 58.3% (n=21/36 combinations; 15/20 SpA, 6/13 PsA) low-activity/remission rate. Of the patients who were receiving glucocorticoids, 55% managed to withdraw them during follow-up. Interestingly, only four serious adverse events in three patients were observed, leading to DTT discontinuation.

[Osteoporosis as a first manifestation of systemic mastocytosis. Study of 6 cases]. / Osteoporosis como primera manifestación de una mastocitosis sistémica. Estudio de 6 casos.

BACKGROUND AND OBJECTIVE: We describe 6 cases of secondary osteoporosis due to systemic mastocytosis diagnosed in the last 6 years. RESULTS: Three females and 3 males, age range: 47-66 years, diagnosed with osteoporosis were subsequently diagnosed with systemic mastocytosis. Diagnosis delay: 0.5-17 years. Cutaneous involvement was present in 3 patients, hematologic involvement in 2 patients and gastrointestinal involvement in 2 patients. Histamine levels in urine were elevated in all cases. Four patients had fractures. Treatment with bisphosphonates was started. After 3 years the values of bone mineral density (BMD) improved in the 5 patients evaluated. Two patients had new vertebral fractures and started teriparatide. CONCLUSIONS: Osteoporosis is an unfrequent initial manifestation of systemic mastocytosis. These patients have a high risk of fractures. Our results suggest that although bisphosphonates improve the bone mineral density the risk of fractures persists.

The SAPHO syndrome: a clinical and imaging study.

The purpose of this study is to describe the clinical and radiological manifestations of patients with the synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome. Retrospective study (1984-2007) was performed in a single center. All patients with the SAPHO syndrome were included. Fifty-two patients were included: 26 male, mean age at diagnosis is 42±12 years. Ostearticular involvement was present before cutaneous involvement in 59.6% of patients and concomitantly in 23.5%. Anterior chest pain was the commonest clinical manifestation, it was present in 38 patients (73%), followed by peripheral arthritis in 17 patients (32%), and sacroliliac pain in 14 patients (26.9%). Cutaneous involvement was present in 33 patients (63.5%). HLA B27 antigen was present in eight patients (17.7%). Bone scintigraphy showed an increased uptake in 42 patients (93.3%). The location of the uptake was mainly in sternoclavicular and manubriosternal joints. CT scan was performed in all "hot joints" showing sclerosis, erosions, hyperostosis, and soft tissue involvement. Refractory patients were treated mainly with pamidronate. Although SAPHO syndrome is an entity that share features that fit into a variety of established disease categories, the present study has a homogenous clinical and radiological pattern that gives support to believe that the SAPHO syndrome is an isolated clinical entity.

Tumoración pétrea paracervical en paciente con esclerosis sistémica forma limitada / Hard paracervical tumor in a patient with limited systemic sclerosis

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Validity, reproducibility, and responsiveness of a twelve-joint simplified power doppler ultrasonographic assessment of joint inflammation in rheumatoid arthritis.

OBJECTIVE: To investigate the validity, reproducibility, and responsiveness of a simplified power Doppler ultrasound (PDUS) assessment of joint inflammation compared with a comprehensive 44-joint PDUS assessment in patients with rheumatoid arthritis (RA) who started therapy with a biologic agent. METHODS: A total of 160 patients with active RA who started a biologic agent were prospectively recruited in 18 Spanish centers. The patients underwent clinical and laboratory assessment and blinded PDUS examination at baseline and 6 months. A PDUS examination of 128 synovial sites in 44 joints was performed. US synovitis and PD signal were semiquantitatively graded from 1 to 3 in all synovial sites. US count and index for synovitis and PD signal were obtained. PDUS intraobserver and interobserver reliability were evaluated. A process of data reduction based on the frequency of involvement of synovial sites by both synovitis and PD signal was conducted. Construct and discriminant validity of a simplified PDUS assessment was investigated. RESULTS: A PDUS simplified assessment including 24 synovial sites from 12 joints detected 100% of patients with synovitis and 91% of patients with PD signal. There was a highly significant correlation between the 44-joint count and index for synovitis and PD signal and the 12-joint count and index for synovitis and PD signal at baseline and 6 months (r = 0.84-0.90, P < 0.0005). The smallest detectable difference was lower than the mean change in simplified PDUS variables. CONCLUSION: A 12-joint PDUS assessment of RA joint inflammation may be a valid, feasible method for multicenter monitoring of therapeutic response to biologic agents.

[Septic pseudopodagra by Streptococcus agalactiae]. / Seudopodagra séptica por Streptococcus agalactiae.

Pseudopodagra is an unusual cause of first metatarsophalangeal arthritis. There are multiple causes, and an infectious cause always has to be excluded. We report a septic pseudopodagra by Streptococcus agalactiae in a patient with chronic hepatopathy with an indolent evolution and a consequent delay in diagnosis. Antibiotic treatment was installed with a favourable outcome without functional sequelae. The pseudopodagra reports in the bibliography are reviewed with special attention on those of infectious aetiology.

Osteoporosis como primera manifestación de una mastocitosis sistémica. Estudio de 6 casos / Osteoporosis as a first manifestation of systemic mastocytosis. Study of 6 cases

Fundamento y objetivo: Se describen 6 casos de osteoporosis como primera manifestación de una mastocitosis sistémica diagnosticados en un período de 6 años. Resultados: Se incluyó a 3 mujeres y 3 varones con edades entre 47 y 66 años. El intervalo entre el diagnóstico de osteoporosis y el de mastocitosis sistémica osciló entre 0,5 y 17 años; 3 presentaban afección cutánea; 2, hematológica y 2, gastrointestinal, y los valores de histaminuria estaban elevados en los 6 casos; 4 pacientes tenían fracturas. Se inició tratamiento con bisfosfonatos. A los 3 años, el valor de densidad mineral ósea (DMO) lumbar mejoró en los 5 pacientes evaluados. A pesar del aumento de DMO, 2 de ellos tuvieron nuevas fracturas vertebrales, por lo que se inició tratamiento con teriparatida. Conclusiones: La osteoporosis como primera manifestación de una mastocitosis sistémica es muy poco frecuente. Suelen ser osteoporosis graves. Aunque los fármacos antirresortivos mejoran la masa ósea en estos pacientes, continúa habiendo riesgo de fracturas

Background and objective: We describe 6 cases of secondary osteoporosis due to systemic mastocytosis diagnosed in the last 6 years. Results: Three females and 3 males, age range: 47-66 years, diagnosed with osteoporosis were subsequently diagnosed with systemic mastocytosis. Diagnosis delay: 0.5-17 years. Cutaneous involvement was present in 3 patients, hematologic involvement in 2 patients and gastrointestinal involvement in 2 patients. Histamine levels in urine were elevated in all cases. Four patients had fractures. Treatment with bisphosphonates was started. After 3 years the values of bone mineral density (BMD) improved in the 5 patients evaluated. Two patients had new vertebral fractures and started teriparatide. Conclusions: Osteoporosis is an unfrequent initial manifestation of systemic mastocytosis. These patients have a high risk of fractures. Our results suggest that although bisphosphonates improve the bone mineral density the risk of fractures persists

Seudopodagra séptica por Streptococcus agalactia / Septic pseudopodagra by Streptococcus agalactiae

La seudopodagra es una causa infrecuente de artritis de primera metatarsofalángica (MTF). Entre sus múltiples causas, siempre hay que descartar la posible causa infecciosa. Se presenta un caso de seudopodagra séptica por Streptococcus agalactiae en un paciente con hepatopatía crónica en el que se retrasó el diagnóstico por su curso indolente. Recibió tratamiento antibiótico intravenoso con buena evolución y sin secuelas funcionales. Se realiza una revisión de los casos de seudopodagra descritos en la bibliografía, con especial atención en los de causa infecciosa(AU)

Pseudopodagra is an unusual cause of first metatarsophalangeal arthritis. There are multiple causes, and an infectious cause always has to be excluded. We report a septic pseudopodagra by Streptococcus agalactiae in a patient with chronic hepatopathy with an indolent evolution and a consequent delay in diagnosis. Antibiotic treatment was installed with a favourable outcome without functional sequelae. The pseudopodagra reports in the bibliography are reviewed with special attention on those of infectious aetiology(AU)

Bursitis séptica por Serratia marcescens / Septic bursitis due to Serratia marcescens

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Síndrome de Bazex, ¿polimialgia reumática o síndrome de cinturas? / Bazex syndrome, polymyalgia rheumatica or shoulder girdle syndrome?

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