Hepatitis B vaccination associated with low response in patients with rheumatic diseases treated with biologics.

BACKGROUND: Hepatitis B virus (HBV) vaccination is recommended for non-immunised patients with rheumatic diseases starting biological disease-modifying antirheumatic drugs (bDMARDs). There is some evidence that HBV vaccination is effective in patients under conventional synthetic disease-modifying anti-rheumatic drugs (csDMARDs), but it is currently unclear whether this also applies to bDMARDs. OBJECTIVES: To assess the efficacy and safety of HBV vaccination in patients with inflammatory arthritides treated with bDMARDs. METHODS: A prospective cohort with inflammatory arthritides treated with bDMARDs, negative for anti-HBs and anti-HBc and never vaccinated for HBV was recruited. Engerix B was administered at 0, 1 and 6 months and anti-HBs was reassessed ≥1 month after last dose. Response was defined as anti-HBs≥10 IU/L and compared against vaccinated healthy controls. Disease flare, serious adverse events and immune-related disorders not previously present were recorded. RESULTS: 62 patients, most treated with TNF inhibitors (TNFi), and 38 controls were recruited. Most patients were taking csDMARDs (67.7%) and were in remission/low disease activity (59.4%). Only 20/62 patients (32.3%) had a positive response to vaccination, in comparison to 36/38 age-matched controls (94.7%, p<0.001). Response was seen in 19/51 patients treated with TNFi (37.3%) and in 1/11 (9.1%) patients treated with non-TNFi (p=0.07), including 1/6 treated with tocilizumab (16.7%). Among TNFi, response rates ranged from 4/22 (18.2%) for infliximab to 8/14 (57.1%) for etanercept. No relevant safety issues were identified. CONCLUSIONS: HBV vaccination response in patients with rheumatic diseases treated with bDMARDs was poorer than expected. Our data reinforce the recommendation for vaccination prior to starting bDMARDs.

Arthritis or maybe not? Pachydermodactyly: the great mimicker of juvenile idiopathic arthritis.

Arthritis in the paediatric population is the hallmark of many rheumatic inflammatory diseases, as well as other cutaneous, infectious, or neoplastic conditions. It can be quite devastating, whereby prompt recognition and treatment of these disorders are essential. However, arthritis can sometimes be mistaken for other cutaneous or genetic conditions leading to misdiagnosis and overtreatment. Pachydermodactyly is a rare and benign form of digital fibromatosis, usually manifested by swelling of the proximal interphalangeal joints of both hands, mimicking arthritis. The authors report a case of a 12-year-old boy with a one-year history of painless swelling of the proximal interphalangeal joints of both hands that was referred to the Paediatric Rheumatology department due to the suspicion of juvenile idiopathic arthritis. The diagnostic work-up was unremarkable, and the patient remained asymptomatic over an 18-month follow-up period. A diagnosis of pachydermodactyly was assumed and no treatment was introduced, given the benign nature of the disorder and absence of symptoms. Therefore, it was possible to safely discharge the patient from the Paediatric Rheumatology clinic.

Life-threatening hypereosinophilic syndrome in a patient with rheumatoid arthritis: a case report.

Hypereosinophilia is unusual in rheumatoid arthritis (RA), but can occur in severe long-lasting disease, especially in patients with extra-articular manifestations and high titers of rheumatoid factor (RF). The association of RA and hypereosinophilic syndrome (HES) remains yet poorly known. We present a case of a 46 years old woman with long-standing untreated RA, that presented to emergency department with severe symptoms of constrictive pericarditis with cardiac tamponade and bilateral pleural effusion, that progressed to cardiac arrest, associated to symmetrical polyarthritis and pruritic erythematous skin papules. She was submitted to urgent pericardial drainage and partial pericardiotomy. Laboratory analyses revealed hypereosinophilia, and elevated inflammatory parameters and immunoglobulin E. The histological study of the pericardium showed results consistent with inflammatory fibrinous pericarditis. Taking into account the presence of some characteristics that are usually present in cases of reactive HES instead of idiopathic HES, and after an intensive diagnostic study, that could rule out other potential causes of secondary HES, the diagnosis of HES associated with RA was made. She started glucocorticoids during hospitalization and methotrexate 15mg per week at the first outpatient rheumatology visit. After 12 weeks of treatment, we considered that she was in clinical and analytical remission, consistently maintaining that after a complete tapering of glucocorticoids. This case illustrates that clinicians should be aware that HES (including severe life-threatening cases) can occur in patients with RA, especially in cases of long-lasting disease with high titters of RF and without treatment, even in the absence of extra-articular features.

The plague: a case of delusional infestation with folie à deux / A praga: um caso de delírio de infestação com folie à deux

Folie à deux or Shared psychotic disorder (SPD) is a rare condition characterized by shared psychotic symptoms between two or more individuals. Delusional parasitosis (DP) is an uncommon psychiatric illness in that patients believe they are infested by insects, without evidence to support this belief. DP occurs in 5­15% of SPD. We report a case of cutaneous DP with SPD between an elderly mother and a daughter that lived together and withdrew from other social contacts for the last three years. We aim to highlight the relationship between SPD and DP, its prognosis, and clinical implications.

Folie à deux ou Perturbação Psicótica Compartilhada (PPC) é uma condição rara caracterizada por sintomas psicóticos compartilhados entre dois ou mais indivíduos. O delírio parasitário (DP) é uma doença psiquiátrica incomum em que os pacientes acreditam estar infestados por insetos, sem evidências que sustentem essa crença. O DP ocorre em 5 a 15% das PPC. Relatamos um caso de um DP cutâneo com PPC entre uma mãe idosa e uma filha que viviam juntas e afastadas de outros contatos sociais nos últimos três anos. O nosso objetivo é destacar a relação entre PPC e o DP, o seu prognóstico e implicações clínicas

Schizoaffective Disorder: How long does it take to diagnose? A case report / Transtorno Esquizoafetivo: Quanto tempo leva para diagnosticar?: "relato de caso"

Introduction: It is reported a case of a 57-year-old woman with multiple psychiatric hospitalizations, during which different diagnostic hypotheses and therapeutic procedures were proposed. Case report: After analyzing the patient's clinical records, the medical team proposed a diagnosis of Schizoaffective Disorder. This disorder presents a high risk of recurrent hospitalizations and high costs associated with therapeutic and follow-up withdrawal, yet there is limited data to assess the post-discharge critical periods. Final considerations: Further research in this area is required to adopt effective therapeutic strategies, reduce the probability of hospital admissions, improve prognosis, and lessen associated financial costs.

Introdução: é relatado o caso de uma mulher de 57 anos com múltiplas hospitalizações psiquiátricas, durante as quais diferentes hipóteses diagnósticas e terapêuticas associadas foram propostas. Relato do caso: Após análise dos registos clínicos, a equipa médica propôs o diagnóstico de Perturbação Esquizoafetiva. Esta Perturbação apresenta um elevado risco de re-internamento, para além do custo associado ao abandono do seguimento clínico e terapêutico. Porém, não existem dados suficientes que avaliem os períodos pós-alta. Consideracoes finais: Portanto, tornam-se necessárias pesquisas mais amplas na área para adotar estratégias terapêuticas eficazes, reduzir a probabilidade de re-internamento, melhorar o prognóstico e minimizar os custos financeiros associados.

Psychosis in Huntington´s Disease. a rare and Under-Investigated Psychiatric Manifestation / Psychosis in Huntington's Disease: uma manifestação psiquiátrica rara e pouco investigada

Introduction: Psychotic symptoms are among the least prevalent and under-investigated psychiatric manifestations (PM) of Huntington's disease (HD). Case report: We herein report a case of a 31-year-old male patient who presented PM with a predominance of negative symptoms, without any significant abnormal movement. HD was diagnosed based on positive DNA analysis and family history. HD imposes longitudinal follow-up through a multidisciplinary approach in order to improve the quality of life and prognosis. Conclusion: This case report highlights the importance of comprehending the PM in the initial presentation of HD so that the diagnosis is not delayed until the onset of motor symptoms.

Introdução: Os sintomas psicóticos estão entre as manifestações psiquiátricas (MP) menos prevalentes e pouco investigadas da doença de Huntington (DH). Relado de caso: Relatamos o caso de um paciente do sexo masculino, 31 anos, que apresentou MP com predomínio de sintomas negativos, sem qualquer movimento anormal significativo. A DH foi diagnosticada com base em uma análise de DNA positiva e na história familiar. A DH impõe um acompanhamento longitudinal por meio de uma abordagem multidisciplinar, a fim de melhorar a qualidade de vida e o prognóstico. Conclusão: Este relato de caso destaca a importância da compreensão das MPs na apresentação inicial da DH, para que o diagnóstico não seja atrasado até ao aparecimento dos sintomas motores

First report of concomitant cryptococcal meningitis and anti-NMDAR encephalitis.

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune disorder, seen most often in young adults and children, triggered by tumors or infections. We report a case of cryptococcal meningitis in a patient with sarcoidosis, presenting prominent neuropsychiatric symptoms, electroencephalographic features of autoimmune encephalitis and positive anti-NMDAR antibodies in the cerebrospinal fluid, raising the hypothesis of an infectious immune-mediated mechanism triggering the production of anti-NMDAR antibodies. Since anti-NMDAR encephalitis is potentially fatal and has significant morbidity, further descriptions of its etiological associations are essential to early identification and prompt treatment.

Erythema elevatum diutinum in Crohn's disease-associated Spondyloarthritis - a rare vasculitis, an unusual association.

Erythema elevatum diutinum is a rare neutrophilic dermatoses with vasculitis, which presents as persistent, symmetrical, purple or brownish papules and nodules, mainly in the extensor surface of the limbs. We describe a case of erythema elevatum diutinum and polyarthritis as initial manifestations of Crohn's disease associated spondyloarthritis. A 51-year-old man, from São Tomé e Príncipe, with previous history of treated tuberculosis and chronic hepatitis B infection, was admitted due to 4 months history of polyarthritis, hyperpigmented papules on the extensor surfaces, occasional episodes of bloody mucous diarrhea and significant weight loss. Histology of the skin showed neutrophilic granulocytes with marked fibrosis and moderate karyorrhexis, consistent with erythema elevatum diutinum. Colonoscopy showed erosions in sigmoid and rectum. Diagnosis of erythema elevatum diutinum secondary to Crohn's disease with associated peripheral spondyloarthritis was assumed. The patient was treated with prednisolone, sulphasalazine, metronidazole, azathioprine and tenofovir with good clinical response. As erythema elevatum diutinum can be secondary to multiple systemic diseases, including rheumatic diseases and inflammatory bowel disease, being aware and recognizing this entity can be of great importance for rheumatologists.

Portuguese recommendations for the use of methotrexate in rheumatic diseases - 2016 update.

BACKGROUND: Methotrexate (MTX) is the first-line drug in the treatment of rheumatoid arthritis (RA) and the most commonly prescribed disease modifying anti-rheumatic drug. Moreover, it is also used as an adjuvant drug in patients under biologic therapies, enhancing the efficacy of biologic agents. OBJECTIVES: To review the literature and update the Portuguese recommendations for the use of MTX in rheumatic diseases first published in 2009. METHODS: The first Portuguese guidelines for the use of MTX in rheumatic diseases were published in 2009 and were integrated in the multinational 3E Initiative (Evidence Expertise Exchange) project. The Portuguese rheumatologists based on literature evidence and consensus opinion formulated 13 recommendations. At a national meeting, the recommendations included in this document were further discussed and updated. The document resulting from this meeting circulated to all Portuguese rheumatologists, who anonymously voted online on the level of agreement with the updated recommendations. RESULTS: Results presented in this article are mainly in accordance with previous guidelines, with some new information regarding hepatitis B infection during MTX treatment, pulmonary toxicity monitoring, hepatotoxicity management, association with hematologic neoplasms, combination therapy and tuberculosis screening during treatment. CONCLUSION: The present recommendations combine scientific evidence with expert opinion and attained desirable agreement among Portuguese rheumatologists. The regular update of these recommendations is essential in order to keep them a valid and useful tool in daily practice.