Revisión clínica, científica, teórica y documental teniendo como objetivo principal realizar una revisión sobre la porfiria aguda, su enfoque, diagnóstico y tratamiento. Se utilizó una metodología descriptiva, cualitativa y teórica que permitió la revisión bibliográfica de los estudios relacionados con la porfiria aguda, trastorno congénito poco frecuente que causa alteraciones en las enzimas de la biosíntesis del grupo hem. La importancia de esta patología radica en que se confunde con otras enfermedades debido al cuadro clínico complejo difícil de identificar, lo que ocasiona un diagnóstico tardío que puede comprometer la vida del paciente y por ello se conoce como enfermedad silenciosa. Como conclusión se recomienda mayor atención a los factores precipitantes de los ataques agudos por la elevada morbimortalidad, así como hacer un diagnóstico rápido y oportuno por medios cualitativos y cuantitativos solicitando PBG (porfobilinógeno) y ALA (ácido delta aminolevulínico).
La anemia hemolítica autoinmune (AHAI) es un trastorno con autoanticuerpos dirigidos contra los glóbulos rojos lo que acorta su supervivencia. Su etiología es diversa, puede ser idiopática o secundaria a consumo de medicamentos o a enfermedades autoinmunes. La fisiopatología exacta sigue sin estar clara en los casos en que se ha descrito secundaria a enfermedad tiroidea, siendo la mayoría de los pacientes hipertiroideos en el momento del diagnóstico de AHAI. Presentamos un caso con antecedente de AHAI idiopática con múltiples exacerbaciones, que ingresa por síntomas sugestivos de tirotoxicosis vs tormenta tiroidea, manejado con metimazol, propanolol, corticoides y colestiramina. Se concluye que el paciente desarrolló una AHAI secundaria a un hipertiroidismo, por lo que es importante evaluar las causas secundarias y realizar un seguimiento estricto debido al componente autoinmune de su enfermedad.
Background:Polypharmacy (PP) is an important risk factor for drug toxicity, delirium, falls, hospitalizations and death. Patients with rheumatoid arthritis (RA) often have comorbid conditions and have PP. Treat to target strategy (T2T) implies a drug escalation and rheumatologists may not apply it in patients with PP.Objectives:Our objective was to analyze if PP affects T2T in a real-world scenario.Methods:Observational, retrospective cohort study. Patients with a new RA diagnosis (ACR/EULAR 2010 criteria) after 2010, over 18 years old, belonging to a Health Management Organization (HMO) from a university tertiary hospital, with a minimum follow-up period of 2 years, were included. PP was defined as consumption greater than or equal to 5 medications at the time of RA diagnosis, regardless of the medication used for RA, administered for a minimum period of 6 months. T2T strategy was defined as accomplished if an escalation in treatment was done when the patient had moderate or high disease activity at medical visit (by DAS28 and/or CDAI), without a significant improvement with respect to the previous visit. Prevalence of PP at RA diagnosis was calculated and RA patients were divided in those with PP at RA diagnosis time and those without. The first 2 years of disease were analyzed and compare between both groups: clinical and demographic characteristics, percentage of visits where T2T was applied, treatments received during that period. A multivariate logistic regression analysis was performed in order to identify factors associated with no T2T compliance.Results:147 patients with RA were included, 86% women, with an average age at diagnosis of 60 years (SD: 15.8). The prevalence of PP at RA diagnosis was 12% (17 patients). Table 1 shows the comparison between patients with and without PP. Patients with PP showed a greater frequency of erosions at baseline and after 2 years of disease, a greater use of corticosteroids at 2 years, higher percentage of hospitalizations and a higher mortality. In the multivariate logistic regression analysis, no compliance of the T2T strategy was only associated with the consumption of corticosteroids at 2 years (OR: 0.36, CI95%: 0.15-0.85; p=0.019) and no association was found with PP at the beginning of the disease.Table 1.Demographic and clinical characteristics in patients with and without polypharmacy.)Patients without Polypharmacy(n= 127)Patients with Polypharmacy(n= 17)PMale sex, n (%)18 (13.8)3 (17.6)0.6Mean age at diagnosis (SD)58.5 (15.6)70.6 (12.2)0.9Positive rheumatoid factor, n (%)87 (66.9)12 (70.5)0.7Positive anti CCP, n (%)109 (85.8)13 (81.2)0.6Erosions at baseline, n (%)25 (19.2)7 (41.1)0.03Active smoker, n (%)28 (21.5)3 (17.6)0.2Past smoker, n (%)23 (17.6)7 (35.2)Charlson score, median (IQR)3 (1-4)4 (4-5)<0.0001Polypharmacy at 2 years, n (%)17 (13.0)46.8 (88.2)<0.0001Rheumatological consultations in 2 years, mean (DE), (IC 95%)8.6 (3.3)(8.0-9.2)8.8 (3.1)(7.2-10.5)0.6Corticosteroid therapy at 2 years, n (%)29 (22.3)8 (47.0)0.02cDMARDs therapy at 2 years, n (%)114 (87.6)13 (76.4)0.2Biologic therapy at 2 years, n (%)13 (10)4 (23.5)0.1Erosions at 2 years, n (%)29 (22.3)9 (52.9)0.007No T2T compliance, n (%)65 (50)5 (29.4)0.1Hospitalizations, n (%)12 (9.2)7 (41.1)<0.0001Mortality, n (%)2 (1.5)2 (11.7)0.01Conclusion:The prevalence of PP in our patients with a new RA diagnosis was 12% and was associated with more baseline erosions, a higher consumption of steroids, and a higher frequency of hospitalizations and mortality during the first 2 years of the disease. No relationship between PP and adherence to the T2T strategy was demonstrated. In the multivariate logistic regression analysis, no compliance with the T2T strategy was only associated to the consumption of corticosteroids at 2 years, may be reflecting a poorer disease control.Disclosure of Interests:JOHN FREDY JARAMILLO GALLEGO: None declared, Javier Rosa: None declared, Marina Scolnik: None declared, Mayra Alejandra Tobar Jaramillo: None declared, LEANDRO FERREYRA: None declared, Enrique Soriano Grant/research support from: AbbVie, Eli Lilly, GlaxoSmithKline, Novartis, Pfizer Inc, Sandoz, Consultant of: AbbVie, Eli Lilly, GlaxoSmithKline, Novartis, Pfizer Inc, Sandoz, Speakers bureau: AbbVie, Amber, Bristol-Myers Squibb, Eli Lilly, Novartis, Pfizer Inc, Roche
Background:Axial spondyloarthritis (SpA) is an umbrella term encompassing a number of inflammatory conditions involving the axial skeleton. A substantial delay between disease onset, diagnosis and treatment often occurs, related in part to under-recognition of SpA symptoms. Although several studies have investigated since the publication of the ASAS classification criteria in 2009, the prevalence and incidence of axial SpA in the general population and in patients with SpA-related conditions, the actual prevalence of SpA in many countries (including Argentina) is unknown.Objectives:To estimate the prevalence of axial SpA and the amount of undiagnosed axial SpA in people under 45 years of age that contacted the health care system for chronic low back pain.Methods:The setting was a university hospital-based health management organization with a population distribution similary to that of Buenos Aires. All electronic medical records of patients < 45 years of age at the time of onset of symptoms (as per the ASAS 2009 criteria) and chronic low back pain for 3 or more months) seen at the university hospital-based health management organization between 2009 and 2019, were reviewed. If the patient fulfilled the ASAS criteria, was classified as having axial SpA [ankylosing spondylitis (AS) or non-radiographic axial SpA (nr-axSpA)]. Among this group, if the diagnosis was already established in the medical records by the treating physicians, these patients were also classified as diagnosed with axial SpA; if not, they were classified as undiagnosed with axial SpA. We are reporting the results of descriptive analysis.Results:A total of 796 patients were included (Table 1), 426 were women (53.52%, 95% CI 50.1-57) with a median age of 34 years (IQR 29-40) at initiation of low back pain with a median follow up of 77.7 months (IQR 35.7-136.4). The prevalence of axial SpA among patients with chronic low back pain was 5.78% (n= 46, 95% CI 4.2-7.4). 22 patients had AS (2.76%, 95% CI 1.6-3.9) with a median lag time between the onset of low back pain and diagnosis of 58.7 months (IQR 33.5 - 92). All AS cases were already diagnosed. 24 patients had nr-axSpA (3.02%, 95% CI 1.8 - 4.2). Of those, 14 were diagnosed by treating physicians with a lag time median of 23.2 months (IQR 13.1 - 36.5) between the onset of low back pain and diagnosis. Ten patients fulfilled the ASAS criteria (41.7%, CI 95% 22 - 61.4) but were not diagnosed by the treating physicians (22%, 95% CI 9.82-33.66) among the patients with axial SpA.Table 1.Demographic, clinical features and therapeutic characteristics of patients with chroniclow back pain stratified by diagnosisAxial spondyloarthritisN=46Ankylosing spondylitisN=22Diagnosednr-axSpAN=14Undiagnosednr-axSpAN=10OtherdiagnosisN=749Female, n (%, CI)18(39.13%, 25.04-53.23)4(18.18%, 2.06-34.29)10(71.42%,47.76-95.09)4(40%, 9.63-70.36)407(54.34,50.77-57.9)Age at chronic LBP initiation, years, median (IQR)36(29.25-40)32(32-40)38(22-36.75)39(35.25-41.5)34(29-40)Follow up, median months (IQR)88(33.43-148.66)33.67(23.38-90.34)16.73(7.64-24.02)64.77(11.21-164.7)77.69(35.83-135.32)Inflammatory chronic LBP by any criteria n (%)44 (95.7)21 (95.5)13 (92.8)10 (100)56 (7.5, 5.6-9.4)Seen by a Rheumatologist, n (%)42 (91.3)22 (100)14 (100)6 (60)36 (5.1)Lag time between first LBP to SpA diagnosis, months, median (IQR)34.6 (22.6-63.2)58.7 (33.5 – 92)23.1 (13.1-36.5)--bDMARDs treatment n, (%, CI)15(33; 19.5–48)10(45; 24.4–67.8)5(36; 13–64.9)--Lag time between NSAIDs failure and first bDMARDs, months, median (IQR)2.66(2.05-4.63)2.76(2.07-11.3).2.66(2.04-3.25)--CI: 95% confidential interval, IQR: interquartile range, bDMARDs: biologic disease modifying anti-rheumaticdrugs, LBP: low back painConclusion:In our cohort, 5.78% of the patients < 45 years with chronic low back pain had axial SpA (AS: 2.76%; and nr-axSpA: 3.02%). Approximately, one in every five patients had undiagnosed axial SpA. Original manuscript made in collaboration with Novartis Argentina S.ADisclosure of Interests:Mayra Alejandra Tobar Jaramillo: None declared, Nicolas Marin Zucaro: None declared, Javier Rosa: None declared, Josefina Marin: None declared, Maria Laura Acosta Felquer: None declared, LEANDRO FERREYRA: None declared, JOHN FREDY JARAMILLO GALLEGO: None declared, Josefina Marcos Employee of: Novartis, Vanesa Duarte Employee of: Novartis, Enrique Soriano Speakers bureau: AbbVie, Amber, Bristol-Myers Squibb, Eli Lilly, Novartis, Pfizer Inc, Roche, Consultant of: AbbVie, Eli Lilly, GlaxoSmithKline, Novartis, Pfizer Inc, Sandoz, Grant/research support from: AbbVie, Eli Lilly, GlaxoSmithKline, Novartis, Pfizer Inc, Sandoz.
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