Sexto reporte de eventos adversos con tratamientos biológicos en Argentina: Informe del registro BIOBADASAR / Sixth report of adverse events with biological treatments in Argentina: Report of the BIOBADASAR registry

Objetivo: Actualizar los resultados del registro BIOBADASAR sobre seguridad, duración y causas de interrupción del tratamiento luego de 8 años de seguimiento. Métodos: BIOBADASAR es un registro de seguridad de terapias biológicas establecido por la Sociedad Argentina de Reumatología. Se presenta la descripción de BIOBADASAR 3.0, una cohorte compuesta por 53 centros de Argentina seguidos prospectivamente desde agosto de 2010 hasta enero de 2018. Resultados: Se registraron 4656 pacientes, 6234 tratamientos [3765 casos (terapia con biológicos) y 2469 controles (terapia no biológicos)]. Se interrumpió el tratamiento en el 44,6% en los casos vs. 27,9% en los controles. Causa principal de discontinuación fue por ineficacia (40% casos vs. 32% controles). Se presentaron 3154 eventos adversos (2230 en casos vs. 924 en controles), de los cuales el 13,6% fueron graves (9,8% en casos y 3,7% en controles). El evento adverso (EA) más frecuente en ambos grupos fueron las infecciones (43,56% en casos vs. 34,31% en los controles, RR: 3,42; IC 95%: 3,02-3,88), y de ellas las de vías aéreas superiores (14,5%). Las neoplasias se presentaron en 78 casos vs. 45 en controles (RR: 1,98; IC 95%: 1,37-2,86). Conclusiones: En este sexto reporte no se observan tendencias diferentes sobre seguridad, duración y causas de interrupción del tratamiento respecto a informes previos. Las infecciones fueron el principal EA y la ineficacia, seguido por EA y la pérdida de pacientes las principales causas de suspensión del tratamiento. El advenimiento de nuevos agentes biológicos y la necesidad de control en seguridad a largo plazo, fortalece el uso de este tipo de registro.

Objective: Update the results of the BIOBADASAR registry on safety, duration and causes of treatment interruption after 8 years of follow-up. Methods: BIOBADASAR is a safety record of biological therapies established by the Argentine Society of Rheumatology. The description of BIOBADASAR 3.0 is presented, a cohort of 53 centers in Argentina followed prospectively from August 2010 to January 2018. Results: 4656 patients were registered, 6234 treatments [3765 cases (therapy with biologicals) and 2469 controls (non-biological therapy)]. Treatment was interrupted in 44.6% in cases vs. 27.9% in controls. Main cause of discontinuation was due to inefficiency (40% cases vs. 32% controls). There were 3154 adverse events (2230 in cases vs. 924 in controls), of which 13.6% were tombs (9.8% in cases and 3.7% in controls). The most frequent adverse event (AE) in both groups were infections (43.56% in cases vs. 34.31% in controls, RR: 3.42, 95% CI: 3.02-3.88), and the upper airway pathways (14.5%). Neoplasms were published in 78 cases versus 45 controls (RR: 1.98, 95% CI: 1.37-2.86). Conclusions: In this article, there are no different trends regarding safety, duration and causes of interruption of treatment compared to previous reports. Infections were the main causes of treatment discontinuation. The advent of new biological agents and the need for control over long-term security, strengthens the use of this type of registration.

Endoscopic endonasal approach to the craniocervical junction: the importance of anterior C1 arch preservation or its reconstruction.

We report our experience with the endoscopic endonasal approaches (EEA) for different craniocervical junction (CCJ) disorders to analyse outcomes and demonstrate the importance and feasibility of anterior C1 arch preservation or its reconstruction. Between January 2009 and December 2013, 10 patients underwent an endoscopic endonasal approach for different CCJ pathologies at our Institution. In 8 patients we were able to preserve the anterior C1 arch, while in 2 post-traumatic cases we reconstructed it. The CCJ disorders included 4 cases of irreducible anterior bulbo-medullary compression secondary to rheumatoid arthritis or CCJ anomalies, 4 cases of inveterate fractures of C1 and/or C2 and 2 tumours. Pre- and postoperative neuroradiological evaluation was always obtained by magnetic resonance imaging (MRI), computed tomographic (CT) scanning and dynamic cranio-vertebral junction x-ray. Pre- and postoperative neurologic disability assessment was obtained by Ranawat classification for patients with rheumatoid arthritis and by Nurick classification for the others. At a mean follow-up of 31 months (range: 14-73 months), an improvement of at least one Ranawat or Nurick classification level was observed in 6 patients, while in another 4 patients neurological conditions were stable. Radiological follow-up revealed an adequate bulbo-medullary decompression in all patients and a regular bone fusion in cases of C1 and/or C2 fractures. In all patients spinal stability was preserved and none required subsequent posterior fixation. The endoscopic endonasal surgery provided adequate exposure and a low morbidity minimally invasive approach to the antero-medial located lesions of the CCJ, resulting in a safe, effective and well-tolerated procedure. This approach allowed preservation of the anterior C1 arch and the avoidance of a posterior fixation in all patients of this series, thus preserving the rotational movement at C0-C2 segment and reducing the risk of a subaxial instability development.

Delayed diagnosed intermuscular lipoma causing a posterior interosseous nerve palsy in a patient with cervical spondylosis: the "priceless" value of the clinical examination in the technological era.

BACKGROUND: Posterior interosseous nerve (PIN) palsy may present with various symptoms, and may resemble cervical spondylosis. CASE REPORT: We report about a 59-year-old patient with cervical spondylosis which delayed the diagnosis of posterior interosseous nerve (PIN) palsy due to an intermuscular lipoma. Initial right hand paraesthesias and clumsiness, together with MR findings of right C5-C6 and C6-C7 foraminal stenosis, misled the diagnostic investigation. The progressive loss of extension of all right hand fingers brought to detect a painless mass compressing the PIN. Electrophysiological studies confirmed a right radial motor neuropathy at the level of the forearm. RESULTS: Surgical tumor removal and nerve decompression resulted in a gradual motor deficits recovery. CONCLUSIONS: A thorough clinical examination is paramount, and electrophysiology may differentiate between cervical and peripheral nerve lesions. Ultrasonography and MR offer an effective evaluation of lipomas, which represent a rare cause of PIN palsy. Surgical decompression and lipoma removal generally determine excellent prognoses, with very few recurrences.

Adaptación al español y validación del cuestionario de detección epidemiológica para artritis reumatoidea / Adaptation and validation to Spanish of rheumatoid arthritis epidemiology detection questionnaire

La detección epidemiológica de la artritis reumatoidea constituye un desafío, dado lo proteiforme de su presentación, por lo que la generación o adaptación y validación de instrumentos es de vital importancia. El objetivo de este estudio es la adaptación lingüística y validación al castellano de un cuestionario utilizado previamente (The prevalence of rheumatoid arthritis in Sweden. Scand J Rheumatol 1999; 28:340-3). Se utilizó una muestra de pacientes con artritis reumatoidea conocida (criterios ACR 87) y controles sanos (N total = 100) para estimar las propiedades de factibilidad, fiabilidad, validez, sensibilidad y especificidad. La sensibilidad del instrumento fue del 100 por ciento (96,9-100), especificidad 94,1 por ciento (88,4-99,7), con un coeficiente Kappa 0,83 (IC95 por ciento: 0,70-0,97) p <0,01. La concordancia entre las preguntas resultó significativa, con Kappa 0,81 (IC95 por ciento: 0,66-0,96) p <0,01 y la consistencia interna mostró un coeficiente de Cronbach 0,892. El análisis factorial exploratorio mostró dosdimensiones coherentes con la versión original.

Systemic lupus erythematosus: mortality and survival in Argentina. A multicenter study.

OBJECTIVE: To analyze the factors associated with mortality, survival and causes of death in patients with systemic lupus erythematosus (SLE) in Argentina. PATIENTS AND METHOD: A series of 366 patients with SLE (45 men and 321 women), mean age 29 y (range 11-70 y) and mean disease duration 6 y, was evaluated from 1990 to 1998. A total of 57 clinical, serological and therapeutic variables were studied. RESULTS: Five- and 10-year survival was 91% and 85% respectively. Forty four patients died (12%): 54% due to sepsis and 32% due to active SLE. Mortality risk factors included heart involvement CRR 3.82), hyperlipidemia (RR 2.72), renal damage (RR 2. 62), infections (RR 2.44), lung disease (RR 2.20) and myositis (RR 2. 07). High-dose prednisone (RR 3.4) or cyclophosphamide (RR 9.19) treatments increased the risk of sepsis (P=0.003) as a cause of death. However, corticosteroids, antimalarial agents and accumulated cyclophosphamide doses proved to be protective factors in overall mortality figures (RR <1). CONCLUSIONS: The main risk factors of death in SLE were heart involvement, hyperlipidemia and renal damage. Treatment with steroids, antimalarial agents and cyclophosphamide improved survival. High-dose corticosteroids and cyclophosphamide were associated with sepsis as a cause of death.

Identification of a regulator that controls stationary-phase expression of catalase-peroxidase in Caulobacter crescentus.

Expression of the catalase-peroxidase of Caulobacter crescentus, a gram-negative member of the alpha subdivision of the Proteobacteria, is 50-fold higher in stationary-phase cultures than in exponential cultures. To identify regulators of the starvation response, Tn5 insertion mutants were isolated with reduced expression of a katG::lacZ fusion on glucose starvation. One insertion interrupted an open reading frame encoding a protein with significant amino acid sequence identity to TipA, a helix-turn-helix transcriptional activator in the response of Streptomyces lividans to the peptide antibiotic thiostrepton, and lesser sequence similarity to other helix-turn-helix regulators in the MerR family. The C. crescentus orthologue of tipA was named skgA (stationary-phase regulation of katG). Stationary-phase expression of katG was reduced by 70% in the skgA::Tn5 mutant, and stationary-phase resistance to hydrogen peroxide decreased by a factor of 10. Like the wild type, the skgA mutant exhibited starvation-induced cross-resistance to heat and acid shock, entered into the helical morphology that occurs after 9 to 12 days in stationary phase, and during exponential growth induced katG in response to hydrogen peroxide challenge. Expression of skgA increased 5- to 10-fold in late exponential phase. skgA is the first regulator of a starvation-induced stress response identified in C. crescentus. SkgA is not a global regulator of the stationary-phase stress response; its action encompasses the oxidative stress-hydrogen peroxide response but not acid or heat responses. Moreover, SkgA is not an alternative sigma factor, like RpoS, which controls multiple aspects of starvation-induced cross-resistance to stress in enteric bacteria. These observations raise the possibility that regulation of stationary-phase gene expression in this member of the alpha subdivision of the Proteobacteria is different from that in Escherichia coli and other members of the gamma subdivision.

Doxifluridine in colorectal cancer patients resistant to 5-fluorouracil (5-FU) containing regimens.

Doxifluridine (5-dFUR) is a fluoropyrimidine derivative, which is preferentially converted to 5-fluorouracil (5-FU) within tumour tissues. Although the activity of 5-FU in metastatic colorectal cancer is well recognised, resistance to this agent is frequently observed and remains its major limitation. The aim of this phase II study was to evaluate the activity of oral and i.v. 5-dFUR in metastatic or locally advanced colorectal cancer patients, who had been previously treated with a 5-FU containing regimen in either an adjuvant or metastatic setting. We treated 48 patients who, on the basis of tumour progression during, or within 8 weeks of the discontinuation of 5-FU therapy, were considered 5-FU resistant, 14 of the patients received 5-dFUR 3000 mg/m2 as a 1-h i.v. infusion, combined with L-leucovorin 25 mg/dose on days 1-5, every 3 weeks; the remaining 34 received oral 5-dFUR 1200 mg/m2 for 5 days followed by 5 days off. Oral L-leucovorin 25 mg/dose was administered 2 h before 5-dFUR. On the basis of WHO criteria, 4/14 (29%, 95% CI 4-51) partial responses were noted in the i.v. treated patients, and 4/34 (12%, 95% CI 1-23) in those treated orally. The radiological examinations documenting the response were a CT scan in 4 cases, ultrasound in 2 and NMR in 2. The median response duration was 6 months (range 3-11+), whereas the median time to treatment failure was 4 months (range 2-17). The responses were achieved in cases previously treated with a median of 9250 mg/m2 (range 5500-18,650) of 5-FU. No CTC-NC1 grade 4 toxicity was observed, although grade 3 diarrhoea occurred in 5 of the orally treated and in 3 of the intravenously treated patients. This is the first report documenting the efficacy of 5-dFUR in patients resistant to 5-FU therapy, and suggests that there is an absence of complete cross-resistance between these two fluoropyrimidines.

Randomized phase II noncomparative trial of oral and intravenous doxifluridine plus levo-leucovorin in untreated patients with advanced colorectal carcinoma.

BACKGROUND: Doxifluridine (5-dFUR) is a fluoropyrimidine derivative that has been shown to be active on a variety of solid tumors. The clinical use of intravenous (i.v.) 5-dFUR as a bolus injection or short term infusion has been limited because of its unpredictable severe neurotoxicity. Unlike fluorouracil (5-FU), 5-dFUR is effective when administered orally. METHODS: This randomized, parallel-group, Phase II trial of two schedules of 5-dFUR was conducted between April 1993 and September 1994. A total of 130 previously untreated patients with locally advanced or metastatic colorectal carcinoma were randomized to receive oral levo-leucovorin (1-leucovorin) 25 mg/dose followed by oral 5-dFUR 750 mg/m2 twice daily for 4 days every 12 days (arm A) or i.v. 1-leucovorin 25 mg/dose followed by i.v. 5-dFUR 3000 mg/m2 for 5 days every 21 days (arm B). RESULTS: The two treatment arms were well balanced in terms of age, sex, and disease extension. Metastases were present in more than 90% of the total population, with the liver being the most common site. A median of 7 oral courses (range, 1-15) and 5 intravenous courses (range, 1-9) were administered. Intent-to-treat analysis rate of the randomized patients revealed a response rate of 15% (95% confidence interval [CI], 7-26) in arm A and 41% (95% CI, 29-54) in arm B. However, 7 cases in arm A and 12 in arm B were inadequately treated, and the response rates, according to standard analysis, were respectively 17% (95% CI, 8-28) and 51% (95% CI, 37-65). The median time to treatment failure was 4 months (range, 1-23) and 7 months (range, 1-9), respectively, for the two groups; median survival was 11 months (range, 1-24) in both groups. National Cancer Institute Grade 3 and 4 diarrhea were observed in 25% of the orally treated patients and in 18% of those receiving i.v. treatment. Stomatitis was reported mainly in arm B (15%). Mild and moderate neurotoxicity was observed in 6% of the patients in both arms; no severe neurotoxicity was reported. CONCLUSIONS: 5-dFUR with l-leucovorin, administered either orally or intravenously, produces response rates that are similar to those offered by the regimens containing 5-FU that are usually used to treat advanced colorectal carcinoma. This study documents the good tolerance of the i.v. schedule administered as a 1-hour infusion; furthermore, oral administration seems to be promising and feasible as a home treatment.

A pilot safety study of capecitabine, a new oral fluoropyrimidine, in patients with advanced neoplastic disease.

5-fluorouracil (5-FU) is still one of the most prescribed cytostatic drugs, but gastrointestinal toxicity limits its use. Capecitabine, an orally administered prodrug of 5-FU, is activated by a cascade of three enzymes, resulting in the preferential release of 5-FU at the tumor site; it was developed in an attempt to avoid the problem of gastrointestinal toxicity of fluoropyrimidines. The aim of the present study was to investigate the safety profile of capecitabine at the daily oral dose of 502 mg/m2, given in two divided doses 12 hr apart for at least 10 days of treatment. In conformity with Italian law, 11 patients (8 females and 3 males) with advanced or metastatic pretreated solid tumors (4 colon-rectum, 3 breast, 2 stomach, 1 ovary, 1 lung) were enrolled. Treatment duration ranged from 1.5 to 14 days. Ten of the 11 patients received the planned 10 days of treatment. One patient was discontinued on the second treatment day when he presented with symptoms of intracranial hypertension with multiple brain metastases documented by CT scan. Toxicity consisted of 1 case of mild edema; no adverse events characteristic of fluoropyrimidines were recorded. No abnormalities in hematologic, renal, hepatic or electrolyte values were seen. In conclusion, capecitabine, given at this dose and for a relatively short period, proved to be well tolerated. Further investigation is recommended to define the promising antitumor efficacy documented in many human xenograft models in mice.

Doxifluridine as palliative treatment in advanced gastric and pancreatic cancer patients.

BACKGROUND: The association of 5-fluorouracil (5-FU) and leucovorin is currently the most used combination in the treatment of advanced gastrointestinal neoplasms. Doxifluridine (d-FUR) is a fluoropyrimidine derivative that is converted into 5-FU inside tumor cells, where it is selectively cytotoxic. The oral administration of dFUR has been extensively investigated in colorectal carcinoma, and has been proven to be active and well tolerated. The purpose of this study was to test the effectiveness of the oral combination with dFUR plus l-leucovorin in gastric and pancreatic cancer patients. METHODS: A total of 50 cases were treated with l-leucovorin 25 mg, followed 2 h later by d-FUR 1,200 mg/m2 for 5 days; the cycles were repeated every 10 days. The regimen was given for a maximum of 36 cycles or until disease progression. Twenty-six patients had gastric cancer (all of whom were pretreated with polychemotherapy) and 24 had advanced pancreatic carcinoma. RESULTS: Objective responses were obtained in 4 (15%; 95% Cl 1-29) patients with gastric cancer, and in 1 (4%) with pancreatic cancer. The median response duration was 4 months. All of the responses were seen in patients previously treated with 5-FU-containing regimens. The median survival in gastric cancer patients was 7 months. Toxicity was moderate: WHO grade III and IV diarrhea was observed in 14% of the cases. CONCLUSIONS: This study indicates the efficacy of oral d-FUR plus l-leucovorin as palliative treatment in gastric cancer patients. The results in pancreatic carcinoma are disappointing but are in line with the published data relating to fluoropyrimidines.

Clinical efficacy of octreotide in the treatment of metastatic neuroendocrine tumors. A study by the Italian Trials in Medical Oncology Group.

BACKGROUND: The unsatisfactory control of neuroendocrine tumor growth with chemotherapy and/or interferon (IFN-2a) stimulated us to investigate the role of the somatostatin analogue octreotide (SMS 201.995), which is reported to be highly effective in controlling carcinoid syndrome symptoms. Octreotide has been used in a wide range of doses, and it was postulated that higher doses might lead to an objective response. METHODS: The aim of the present multicenter Phase II study was to determine the safety and efficacy of SMS 201.995 in controlling carcinoids and other neuroendocrine tumors. Fifty-eight patients were treated subcutaneously with 2 sequential doses of the drug (Sandostatina, Sandoz, Inc., S.b.A. Pharmaceuticals, Basel, Switzerland). The first 23 patients received 500 micrograms 3 times a day and the remaining 35 patients received 1000 micrograms 3 times a day. The treatment was continued until the tumor progressed. RESULTS: All of the patients were adequately treated and evaluated. The predominant histotype was carcinoid, although there were instances of medullary thyroid carcinoma, pancreatic islet cell tumors, and Merkel cell carcinoma. Carcinoid syndrome was documented in 16 patients and abnormal urinary 5-hydroxyindoloacetic acid excretion in 15. The median treatment duration was 5 months (range, 2-31 months). The responses were evaluated in three categories: tumor regression for tumor growth control, symptom response, and biochemical response. There was an effect on tumor growth in two patients with carcinoids. Symptomatic control was achieved in 73% of patients and a biochemical response in 77% of patients. In twenty-seven patients, the disease stabilized for at least 6 months (range, 6-32+). The median survival time for all patients was 22 months (range, 1-32+). CONCLUSIONS: In terms of tumor regression, octreotide is disappointing (partial response: 3%); symptomatic response and biochemical control are satisfactory. These data confirm that somatostatin analogues are comparable to interferons in the treatment of carcinoid syndrome, although other efforts are necessary to control tumor regression.

Doxifluridine and leucovorin: an oral treatment combination in advanced colorectal cancer.

PURPOSE: This study was designed to test the activity and feasibility of an all-oral regimen of levo-leucovorin and doxifluridine (dFUR) in the treatment of advanced colorectal cancer and to establish whether the pharmacokinetics of dFUR and fluorouracil (FU) are affected by demographic and/or biologic parameters. MATERIALS AND METHODS: One hundred eight patients with histologically proven colorectal cancer received orally administered levo-leucovorin 25 mg followed 2 hours later by dFUR 1,200 mg/m2 on days 1 to 5, with the cycle being repeated every 10 days. RESULTS: Among 62 previously untreated patients, two complete responses (CRs) and 18 partial responses (PRs) were observed (overall response rate, 32%; 95% confidence interval, 21% to 45%). The median response duration was 4 months (range, 2 to 13) and the median survival time, 14 months. Among 46 pretreated patients, there were three CRs and three PRs (response rate, 13%; 95% confidence interval, 5% to 26%). In this group of patients, the median response duration was 4 months (range, 1 to 12) and the median survival time, 12 months. No toxic deaths were observed. The only World Health Organization (WHO) grade 3 to 4 side effect was diarrhea (32 patients). CONCLUSION: This regimen is active in previously untreated colorectal cancer patients and combines good compliance with safety. Limited but definite efficacy was also detected in the patients previously treated with FU, which suggests incomplete cross-resistance between the two drugs. The pharmacokinetic results suggest that the conversion rate of dFUR to FU increases between days 1 and 5, but that FU levels remain low in comparison to those measured after classical FU therapy. Under the experimental conditions used in this study, the interpatient variability of pharmacokinetic parameters remains largely unexplained by the tested variables.

Doxifluridine: an active agent in advanced gastrointestinal cancer.

BACKGROUND: The results of the use of chemotherapy in patients with advanced gastrointestinal malignancies have been disappointing. Complete responses are rare, and even partial responses are generally few with no benefit on survival. Since its introduction in clinical trials more than 30 years ago, fluorouracil has remained the most effective single agent in the treatment of these diseases. Doxifluridine is a new fluoropyrimidine derivative, that is converted into fluorouracil, its active component. Experimental data confirm cytotoxic selectivity for human tumor cells. METHODS: Three trials have been conducted at the Istituto Nazionale Tumori of Milan, to evaluate the tolerability and efficacy of doxifluridine administered endovenously or orally in patients affected by different gastrointestinal neoplasms. The data will be discussed. CONCLUSIONS: Our results indicate that doxifluridine may be superior to fluorouracil, the biochemical modulator as folinic acid seems to enhance its activity in colorectal patients. The oral schedule is feasible for home treatment.

A phase II trial of dacarbazine, fluorouracil and epirubicin in patients with neuroendocrine tumours. A study by the Italian Trials in Medical Oncology (I.T.M.O.) Group.

BACKGROUND: Previous experiences in the treatment of neuroendocrine tumours have demonstrated some activity of single agents such as adriamycin, fluorouracil (FU), streptozotocin and dacarbazine (DTIC). Opinions concerning the usefulness of polychemotherapy in carcinoid tumours are discordant, whereas better results have been achieved in other endocrine pancreatic neoplasms. Based on this background, we used multidrug chemotherapy with DTIC, FU and epirubicin in the treatment of different neuroendocrine tumours. METHODS: The study involved 38 pts with progressive and measurable disease. The treatment schedule was FU 250 mg/m2 i.v., epirubicin 25 mg/m2 i.v., and DTIC 200 mg/m2 i.v. on days 1, 2 and 3 every 3 weeks. RESULTS: The responses achieved by histologic types were carcinoids 2/20, medullary thyroid carcinoma 1/7, neuroendocrine tumours 1/6; and Merkel cell carcinoma 3/5. The median duration of response was 5 months (range 2-11). Stable disease was observed in 13 cases (34%). Out of the 18 cases in progression, 17 had not responded to previous medical treatment. No symptom control was observed in 4 pts with carcinoid syndrome. Treatment toxicity was moderate and included nausea and vomiting, alopecia, leukopenia and mucositis. CONCLUSIONS: Our results document the moderate efficacy of the regimen in all of the histologic types. The major difference in comparison with previous studies was the lower response rate observed in patients with neuroendocrine tumours.

Tarsal ankylosis in juvenile and adult onset rheumatoid arthritis.

It has been suggested that tarsal arthritis with ankylosis may be characteristic of late onset Still's disease. In our study 16 of 88 (18%) patients with juvenile rheumatoid arthritis (JRA) and 22 of 97 (23%) patients with adult RA had radiographic findings of tarsal joint ankylosis. In our patients, tarsal bony fusion was not related to type of disease onset, age of onset or sex; rather it was related to a longer duration of disease. Tarsal ankylosis should not be regarded as a special feature of Still's disease since it can also be seen in adult RA.

Enfermedad de Still en el niño y en el adulto: estudio comparativo de sus características clínicas; serológicas y de histocompatibilidad / Still disease in children and adults: comparative study of its clinical, serological and histocompatibility characteristics

Diecisiete pacientes con enfermedad de Still de comienzo juvenil y 6 pacientes con comienzo en la edad adulta fueron analizados comparativamente con el objeto de determinar la influencia de la edad en la expresión clínica de esta afección. Doce de 17 pacientes juveniles y 3 de 6 adultos eran de sexo masculino. Fiebre héctica, rash cutáneo y poliartritis se observaron en todos los pacientes de ambos grupos. Leucocitosis se observó en 13 de 17 pacientes juveniles (76%) y en 5 de 6 (83%) pacientes adultos. Pericarditis, esplenomegalia, adenomegalias, anemia y anquílosis ósea se observaron con mayor frecuencia, aunque no significativamente, en el grupo de pacientes de comienzo juvenil. El antígeno HLA DR4 se encontró en 6 de 11 (54,5%) pacientes juveniles, frecuencia significativamente aumentada con respecto a la población general. En los adulto 1 de 6 tenían HLA DR4. No existió relación entre pronóstico favorable y presencia del antígeno HLA Bw35. En este trabajo no se encontraron diferencias significativas en las manifestaciones clínicas y serológicas entre pacientes juveniles y adultos con enfermedad de Still

Enfermedad de Still en el niño y en el adulto: estudio comparativo de sus características clínicas; serológicas y de histocompatibilidad / Still disease in children and adults: comparative study of its clinical, serological and histocompatibility characteristics

Diecisiete pacientes con enfermedad de Still de comienzo juvenil y 6 pacientes con comienzo en la edad adulta fueron analizados comparativamente con el objeto de determinar la influencia de la edad en la expresión clínica de esta afección. Doce de 17 pacientes juveniles y 3 de 6 adultos eran de sexo masculino. Fiebre héctica, rash cutáneo y poliartritis se observaron en todos los pacientes de ambos grupos. Leucocitosis se observó en 13 de 17 pacientes juveniles (76%) y en 5 de 6 (83%) pacientes adultos. Pericarditis, esplenomegalia, adenomegalias, anemia y anquílosis ósea se observaron con mayor frecuencia, aunque no significativamente, en el grupo de pacientes de comienzo juvenil. El antígeno HLA DR4 se encontró en 6 de 11 (54,5%) pacientes juveniles, frecuencia significativamente aumentada con respecto a la población general. En los adulto 1 de 6 tenían HLA DR4. No existió relación entre pronóstico favorable y presencia del antígeno HLA Bw35. En este trabajo no se encontraron diferencias significativas en las manifestaciones clínicas y serológicas entre pacientes juveniles y adultos con enfermedad de Still (AU)