A feasibility study of a short lung ultrasound protocol as a screening method for fibrotic interstitial lung disease , Sara Braga, André Carvalho, , Márcio Rodrigues, , Pedro Madureira, Raquel Miriam Ferreira, , André Terras Alexandre, , Natália Melo, , Patrícia Caetano Mota, , António Morais, , Hélder Novais Bastos, and Arp Rheumatology, 2025 Aims: Interstitial lung disease (ILD) represents a common complication of connective tissue diseases (CTD). Early detection of ILD is critical since patients often remain asymptomatic during the initial stages. While numerous studies have proposed various ultrasound protocols for ILD evaluation, we find these protocols to be time-consuming in our clinical practice. The aim of this study is to introduce a concise lung ultrasound protocol for the screening of pulmonary fibrosis. Methods: We conducted a prospective observational pilot study involving 28 patients followed in consultation for ILDs. By implementing this streamlined protocol, we established correlations between ultrasound findings at specific locations and their corresponding tomographic patterns. Following the assessment of the protocol's diagnostic accuracy, a multidisciplinary meeting was convened to scrutinize ultrasound images from several patients and evaluate the level of agreement among rheumatologists, pulmonologists, and radiologists. Results: Our simplified protocol revealed significant correlations between ultrasonographic pleural irregularity, discontinuity and the number of B-lines, and the tomographic findings of reticulation and honeycombing in both the upper and lower lung regions. This protocol demonstrated high sensitivity and positive predictive value in identifying reticulation and honeycombing, as well as remarkable specificity and negative predictive value for the latter. The results obtained between the specialties showed strong agreement. Conclusion: Preliminary results suggest a role of ultrasound to detect peripheral manifestations of fibrotic ILDs, such as reticulation and honeycombing. This assessment protocol can be adopted effectively by clinicians, such as rheumatologists, to evaluate lung involvement by CTDs. A larger study is necessary to achieve more robust and generalizable results as a screening tool.
An Uncommon Presentation of Secondary Syphilis Rafaela Nicolau, Daniela Oliveira, João Pacheco, Pedro Madureira Journal of Rheumatology, 2024 Syphilis is a chronic venereal disease caused by Treponema pallidum (TP). Destructive arthritis is rare in its primary and secondary stages but can occur in the tertiary stage and its congenital form.
Severe infections in Portuguese patients with rheumatoid arthritis under biologic treatment – a multicenter, nationwide study (SIPPRA-B Study) Arp Rheumatology, 2023
Possible relationship between hydroxychloroquine and electrocardiographic and echocardiographic abnormalities in patients with inflammatory rheumatic diseases––a monocentric study Filipe Oliveira Pinheiro, Miguel Martins Carvalho, Pedro Madureira, Maria Seabra Rato, Filipe Macedo, Lúcia Costa Lupus, 2023 Objective Hydroxychloroquine (HCQ) is used in the treatment of inflammatory rheumatic diseases and is considered a safe drug. The role of HCQ in the COVID-19 pandemic highlighted some deleterious cardiac effects of HCQ. We aim to evaluate the prevalence and development of cardiac-adverse events in HCQ-treated patients with inflammatory rheumatic diseases. Methods We performed a cross-sectional study where patients aged ≥18 years with a diagnosis of inflammatory rheumatic disease currently exposed or not to hydroxychloroquine underwent electrocardiogram (ECG) and echocardiogram. Comparisons between groups were evaluated using chi-square, t test, and Mann-Whitney U test. Logistic regression was performed to determine predictors of changes in ECG and echocardiography. Results Eighty patients were included, 75 (93.8%) female, aged 52 ± 13 years. ECG changes were seen in higher proportion in patients with hypertension (40.6% vs 12.5%, p = .004) and higher median potassium levels—4.5 (4.1–4.8) versus 4.2 (4.0–4.4), p = .023. Echocardiography changes were seen in older patients (59 ± 11 vs 50 ± 13 years, p = .003) and in patients with higher cumulative dose—1752 (785–2190) versus 438 (328–1022) g, p = 0.008 – and time of exposure to HCQ – 12 (6–15) versus 4 (2–9) years, p = 0.028. HCQ cumulative dose (OR 1.001, CI95% 1.000–1.002, p = .033) and exposure time (OR 1.136, CI95% 1.000–1.289, p = .049) were predictors of echocardiography changes, but when adjusted for age, neither HCQ cumulative dose nor exposure time were predictors of echocardiography changes. Conclusion No association was found between changes in ECG and echocardiogram in patients under HCQ, which remains a safe drug in patients with inflammatory rheumatic diseases.
Tuberculous osteomyelitis of the pubic symphysis – a case report of a rare entity mimicking spondyloarthritis Arp Rheumatology, 2023
Oculomotor nerve palsy, an unusual onset of polyarteritis nodosa Ana Martins, Filipe Oliveira Pinheiro, Sofia Vedor, Daniela Oliveira, Maria Rato, Diogo Fonseca, Pedro Madureira, Luís Braz, Sofia Pimenta, Lúcia Costa Reumatologia, 2023 IntroductionCranial nerve involvement in polyarteritis nodosa(PAN) is underrecognized and rarely reported. The aim of this article is to review the available literature and present an example of oculomotor nerve palsy in the course of PAN.Material and MethodsEvaluation of texts describing the analyzed problem using the terms “polyarteritis nodosa”, “nerve”, “oculomotor”, “cranial nerve” and “cranial neuropathy” for searching the PubMed database was done. Only full-text articles in English language with titles and abstracts were included in the analysis. As a guideline for the analysis of articles, the methodology described in the Principles of Individual Patient Data systematic reviews (PRISMA-IPD) was used.ResultsAfter screening articles only 16 reported cases of PAN with cranial neuropathy were included in the analysis. In 10 the cranial neuropathy was reported as the initial manifestation of PAN with optic nerve involvement as the most frequent (62.5%); among these cases the oculomotor nerve was in-volved in 3 cases. Treatment with glucocorticosteroids and cyclophosphamide was the most com-mon.ConclusionsAlthough cranial neuropathy, especially oculomotor nerve palsy is a rare first neurological manifesta-tion of PAN, this clinical problem should be considered in the differential diagnosis. Especially patients with peripheral neuropathy, general symptoms, skin lesions and hepatitis B virus infection should be evaluated for cranial nerve involvement in the course of vasculitis. In the case of unclear involvement of the cranial nerves, PAN should also be considered in the differential diagnosis as the cause of symptoms and the first manifestation of the disease.
Tophi - An Exceptional Cause of Knee Locking Filipe Oliveira Pinheiro, Pedro Madureira Journal of Clinical Rheumatology, 2022 From the Rheumatology Department, Centro Hospitalar Universitário de São João, Porto, Portugal. The authors declare no conflict of interest. Correspondence: Filipe Oliveira Pinheiro, MD, Rheumatology Department, Centro Hospitalar e Universitário de São João, Alameda Prof. Hernâni Monteiro, 4200-319 Porto, Portugal. E-mail: filipe.pinheiro92gmail.com.
Mixed connective tissue disease and severe renal disease - an apparent misconception? Filipe Pinheiro, Pedro Madureira, Maria Rato, Lúcia Costa Reumatologia, 2022 Dear Editor, Mixed connective tissue disease (MCTD) is a connective tissue disease characterized by the presence of anti-RNP (anti-U1 ribonucleoprotein) antibodies, and by a set of signs and symptoms that it shares with other connective tissue diseases, such as systemic lupus erythematosus (SLE) [1]. Because of this clinical overlap of syndromes, the presence of high titer anti-RNP antibodies is a central feature of the diagnosis of MCTD [2]. Importantly, one of the most distinctive features of MCTD is the absence of severe renal or central nervous system involvement, which may be present in other connective tissue diseases, such as SLE, and this is a focal and key point of MCTD [3]. In fact, high anti-RNP titers appear to be protective against renal involvement, particularly diffuse proliferative glomerulonephritis, even when they occur at lower titers, in other connective tissue diseases [4]. Renal involvement mandating intense immunosuppressive therapy has been reported seldomly [3]. Mixed connective tissue disease treatment is largely empirical, since there have been no randomized controlled trials on the treatment of MCTD and no specific guidelines exist either [5]. We report a case of a female patient, 32 years old, who presented with a 2-month history of polyarthritis. Laboratory tests showed a high erythrocyte sedimentation rate (ESR, 69 mm/h), antinuclear antibodies (ANA) with a 1 : 1280 titer, speckled pattern, and strongly positive anti-U1 RNP antibodies. There was no complement decrease, and the results of other immunological testing, comprising anti-double stranded DNA (anti-dsDNA), rheumatoid factor, antineutrophil cytoplasmic antibodies (ANCA), and other autoantibodies to extractable nuclear antigens (ENAs), were normal. The diagnosis of MCTD was made and the patient was started on hydroxychloroquine 400 mg/day and a short course of low-dose steroids. The patient then missed follow-up appointments due to being pregnant. When the patient presented again, she had abandoned all her medications due to pregnancy-related concerns, and had fatigue, new onset of Raynaud phenomenon and puffy hands. Blood tests revealed anemia (hemoglobin 10.3 g/dl) high ESR (97 mm/h), and hypoalbuminemia (32 g/l), and repeated immunological testing was remarkable only for high ANA and anti-RNP antibodies. Urine studies showed proteinuria of 2.5 g/24 h and microscopic hematuria (142/μl). The patient underwent renal biopsy, which showed “segmental sclerosis lesions and presence of mesangial and membrane immune-type deposits by immunofluorescence”, with a full-house pattern on immunofluorescence but absence of mesangial or endocapillary proliferation. Therapy with prednisolone 1 mg/kg/day (60 mg/day) in a progressive dose reduction regimen, hydroxychloroquine 400 mg/day, mycophenolate mofetil (titrating dose up to 3000 mg/day), and captopril was started. At 6 months, prednisolone was reduced to 7.5 mg/day, and she was in renal remission, as renal function was normal, proteinuria had decreased to 370 mg/24 h, serum albumin was normal, and hematuria had resolved. At 24 months after this admission, prednisolone was suspended, and the patient remains in remission. Anti-RNP antibodies are associated with a protective role in MCTD and in other systemic connective tissue diseases where they are present, namely regarding severe renal or central nervous system involvement [4]. Renal disease in MCTD has been described, but is usually in the form of low grade proteinuria or hematuria; in fact,
Recurrent lupus pericarditis treated with anakinra – a case report Arp Rheumatology, 2022
Reuma.pt/vasculitis - The Portuguese vasculitis registry Cristina Ponte, Nikita Khmelinskii, Vítor Teixeira, Karine Luz, Daniela Peixoto, Marília Rodrigues, Mariana Luís, Lídia Teixeira, Sandra Sousa, Nathalie Madeira, Joana A. Aleixo, Teresa Pedrosa, Sofia Serra, Raquel Campanilho-Marques, Walter Castelão, Ana Cordeiro, Inês Cordeiro, Sílvia Fernandes, Carla Macieira, Pedro Madureira, Armando Malcata, Romana Vieira, Fernando Martins, Graça Sequeira, Jaime C. Branco, Lúcia Costa, José Vaz Patto, José Canas da Silva, José A. Pereira da Silva, Carmo Afonso, Helena Canhão, Maria J. Santos, Raashid A. Luqmani, João E. Fonseca Orphanet Journal of Rare Diseases, 2020
Silent acute myocarditis in eosinophilic granulomatosis with polyangiitis Acta Reumatologica Portuguesa, 2018
Sarcoidosis: An unusual presentation Pedro Madureira, Sofia Pimenta, Hélder Cardoso, Rui Guimarães Cunha, Lúcia Costa Reumatologia Clinica, 2017
