scholarly journals AB1045 CLINICAL, ANALYTICAL AND RADIOLOGICAL CHARACTERISTICS OF A COHORT OF PATIENTS WITH SARCOIDOSIS.

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1814.2-1814
Author(s):  
I. Madroñal García ◽  
C. Aguilera Cros ◽  
L. Mendez Diaz
Keyword(s):  
Systemic Disease ◽  
Immunosuppressive Treatment ◽  
Clinical Manifestations ◽  
Corticosteroid Treatment ◽  
Pulmonary Involvement ◽  
List Type ◽  
Chi Square ◽  
Course Of Disease ◽  
Chronic Course ◽  
Radiological Stage

Background:Sarcoidosis is a systemic disease whose etiology is unknown. It is characterized by the formation of granulomas in any tissue of the organism. Ganglionic, pulmonary and cutaneus involvement is the most prevalent.Objectives:Describe clinical characteristics of a cohort of patients with sarcoidosis diagnosed.Define the association between the ACE’s number at diagnosis, radiological lung stage, treatment and course of disease.Evaluate if the extrapulmonary involvement is related to the course of the disease.Methods:Descriptive retrospective study of patients with S diagnosis treated in our Hospital in 2019. Data were obtained by reviewing medical records. Chi-square tests and parametric tests have been used to establish the differences described in the objectives.Results:102 patients diagnosed with sarcoidosis have been included, (51% females) with an average age of 56±11 years. Suspected diagnosis at the onset of disease was S in 70.6% of patients, followed by suspected lymphoma (20.6%). The average time for the definitive diagnosis of S was 9.5 months. 70.6% of the patients had elevated ACE titles at the beginning. Regarding the clinical manifestations, 18.6% of the patients presented fever at the beginning and 66.7% extrathoracic clinical manifestations. 72.5% have lymph node adenopathies, and in 91% there is thoracic involvement (the most frequent pulmonary stage is stage II). A biopsy was performed in 84.3% of the patients, the lung biopsy being the most performed (52.3%). 88.2% of patients received corticosteroid treatment at the onset of the disease (currently under treatment with corticosteroids 37.3%). 50% of patients are treated with immunosuppressants, Methotrexate was the most used. 5 patients are treated with biological therapy (AntiTNF).Regarding the course of the disease, 51% of the patients have a chronic course, 45.1% are in remission and 3.9% have suffered a relapse of the disease. In this study, no significant relationship was found between the ACE values at the onset of the disease, the pulmonary stage and the course of the disease.According to our data, patients presenting with extrathoracic clinical manifestations need more frequently corticosteroid treatment (p = 0.017) and immunosuppressive treatment (p = 0.001) with respect to patients who do not have an extrathoracic clinic. In addition, patients with an extrathoracic clinic present more frequently a chronic course of the disease than those who do not (p = 0.019).Conclusion:The results described in this study are similar to those found in the literature. The differences found can be explained because patients presenting with extrathoracic clinical manifestations have a more complicated management and need more treatment than those with only pulmonary involvement, even patients with radiological stage I do not usually need treatment, only surveillance.Disclosure of Interests:None declared

scholarly journals AB1046 JOINT MANIFESTATIONS IN A COHORT OF PATIENTS WITH SARCOIDOSIS IN A THIRD LEVEL HOSPITAL.

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1814.3-1814
Author(s):  
I. Madroñal García ◽  
C. Aguilera Cros ◽  
L. Mendez Diaz
Keyword(s):  
Medical Records ◽  
Unknown Etiology ◽  
Joint Involvement ◽  
List Type ◽  
Chi Square ◽  
Exact Test ◽  
Approximate Result ◽  
Chronic Course ◽  
Level Hospital ◽  
Larger Sample

Background:Sarcoidosis (S) is a systemic granulomatous disease of unknown etiology, which most frequently affects the ganglion, lung and skin, although it can affect other organs, including the musculoskeletal system.Objectives:- Describe the clinical, analytical and radiological characteristics of patients diagnosed with S presenting joint manifestations.- To assess the association between patients who have joint manifestations and the use of corticosteroids (C) and immunosuppressants (IS), with respect to those without joint involvement.Methods:Retrospective descriptive study of patients with diagnosis of S with joint manifestations, treated in our Hospital from 2017 to 2019. Data were obtained by reviewing medical records. Chi square tests and Fisher’s exact test have been performed to establish the differences described in the objectives.Results:From a database of 102 patients with S, 18 presented joint manifestations (50% women), with a mean age of 57 ± 6 years. Of these patients, 4 (22.2%) have presented positive ANA. Regarding the clinic, 3 patients presented the association of polyarthritis and bilateral ankle swelling, 8 patients presented with polyarthritis, 3 monoatritis and 4 patients presented bilateral ankle swelling. 61.1% had fever at the onset of the disease.14 patients (77.8%) had high ACE values at the onset of the disease, without presenting significant differences with respect to all patients diagnosed with S who do not have joint involvement.All patients received treatment with C and 10 patients (55.5%) needed an IS treatment, finding no differences with respect to patients who do not have joint involvement (p=0.92).On the course of the disease, the majority of patients with joint involvement have a chronic course (72.2%). Nor were significant differences found when compared with patients who have no joint involvement (p = 0.73).Conclusion:Patients with joint involvement in our study have been 17.6% (18), an approximate result to that described in the literature (over 10%), although our result may be increased by the fact that the patients who are followed in Rheumatology present or have presented joint involvement. No significant differences were found between patients with S who presented joint involvement and those who did not, with respect to the initial ACE values, treatment and disease course. Prospective, multicenter and larger sample studies are necessary to better understand these associations.Disclosure of Interests:None declared

scholarly journals Histologically confirmed isolated IgG4-related hypophysitis: two case reports in young women

2014 ◽  
Vol 2014 ◽  
Author(s):  
Gabriela Alejandra Sosa ◽  
Soledad Bell ◽  
Silvia Beatriz Christiansen ◽  
Marcelo Pietrani ◽  
Mariela Glerean ◽  
...  
Keyword(s):  
Young Women ◽  
Plasma Cells ◽  
Case Reports ◽  
Clinical Manifestations ◽  
Corticosteroid Treatment ◽  
Older Men ◽  
Pituitary Stalk ◽  
Symptom Improvement ◽  
List Type ◽  
Sellar Mass

Summary IgG4-related hypophysitis is a recently described entity belonging to the group of IgG4-related diseases. Many other organs can also be affected, and it is more common in older men. To date, 32 cases of IgG4-related hypophysitis have been reported in the literature, 11 of which included confirmatory tissue biopsy and the majority affecting multiple organs. The aim of this report is to present two cases of biopsy-proven IgG4-related hypophysitis occurring in two young female patients with no evidence of involvement of other organs at the time of diagnosis. Learning points IgG4-related hypophysitis belongs to the group of IgG4-related diseases, and is a fibro-inflammatory condition characterized by dense lymphoplasmacytic infiltrates rich in IgG4-positive plasma cells and storiform fibrosis. It is more common in older men, but young women may also present this type of hypophysitis. Although involvement of other organs is frequent, isolated pituitary disease is possible. Frequent clinical manifestations include anterior hypopituitarism and/or diabetes insipidus. The diagnosis may be confirmed with any of the following criteria: a pituitary biopsy with lymphoplasmacytic infiltrates, with more than ten IgG4-positive cells; a sellar mass and/or thickened pituitary stalk and a biopsy-proven involvement of another organ; a sellar mass and/or thickened pituitary stalk and IgG4 serum levels >140 mg/dl and sellar mass reduction and symptom improvement after corticosteroid treatment. Glucocorticoids are recommended as first-line therapy.

Hashimoto encephalopathy

2013 ◽  
Vol 154 (33) ◽  
pp. 1312-1316 ◽  
Author(s):  
Gábor Pocsay ◽  
Andrea Gazdag ◽  
József Engelhardt ◽  
István Szaniszló ◽  
Zoltán Szolnoki ◽  
...  
Keyword(s):  
Psychiatric Symptoms ◽  
Immunosuppressive Treatment ◽  
Unknown Origin ◽  
Corticosteroid Treatment ◽  
Thyroid Peroxidase ◽  
Thyroid Autoantibodies ◽  
Thyroglobulin Antibodies ◽  
Hashimoto Encephalopathy ◽  
Generalized Seizures

The authors present a case report and review the literature on Hashimoto encephalopathy. The onset of the disease may be marked by focal and then progressively generalized seizures or other neurological symptoms, but a cognitive decline or various psychiatric symptoms may also emerge. High levels of anti-thyroid peroxidase antibodies and/or anti-thyroglobulin antibodies are present in the serum. Corticosteroid treatment usually results in an improvement of symptoms. The syndrome is frequently overlooked and, therefore, the authors strongly recommend testing serum thyroid autoantibodies in cases with encephalopathy of unknown origin independently on the presence of thyroid disease in the patient or family history. The importance of long-term immunosuppressive treatment should also be stressed. Orv. Hetil., 2013, 154, 1312–1316.

Apresentação e Evolução Atípicas da Granulomatose de Wegener: Relato de Caso/Atypical Presentation and Evolution of Wegener's Granulomatosis: Case Report

1970 ◽  
Vol 5 (3) ◽  
pp. 53-67
Author(s):  
Aline Dos Santos ◽  
Ana Caroline Balducci Scafi ◽  
Luciene Azevedo Morais ◽  
Pablo Girardelli Mendonça Mesquita
Keyword(s):  
Renal Failure ◽  
Renal Function ◽  
Case Report ◽  
Chronic Renal Failure ◽  
Early Diagnosis ◽  
Wegener’S Granulomatosis ◽  
Immunosuppressive Treatment ◽  
Clinical Manifestations ◽  
Wegener's Granulomatosis ◽  
Creatinine Concentration

RESUMOIntrodução: A Granulomatose de Wegener (GW) é uma vasculite rara e idiopática associada à presença do anticorpo Anticitoplasma de Neutrófilo (ANCA) que acomete, preferencialmente, os pequenos vasos. As manifestações clínicas são diversas, ocorrendo em mais de 90% dos casos, sintomas do trato respiratório. O comprometimento renal é tardio e preditor de mau prognóstico.  Sua morbidade a médio e longo prazo inclui insuficiência renal crônica. A probabilidade de sucesso de manutenção da função renal depende da concentração sérica de creatinina ao início do tratamento, o que indica a importância do diagnóstico e terapêutica adequada precoces. Casuística: Relata-se o caso de uma paciente do sexo feminino, 61 anos, portadora de GW com comprometimento renal avançado à apresentação não precedido por sintomas pulmonares esperados. O tratamento imunossupressor associado a plasmaferese permitiu a melhora da função renal da paciente poupando-a de tornar-se dialítica- dependente. Discussão: A paciente iniciou a doença através de insuficiência renal assintomática, com valores de função renal compatíveis com o estágio mais avançado de doença renal crônica, ultrassonografia dos rins sem alterações compatíveis e sem os sintomas respiratórios esperados. Segundo a literatura, a combinação de imunossupressores e plasmaferese associa-se à recuperação renal em três meses com sobrevivência sem necessidade de diálise por 12 meses, no caso relatado, obteve-se tal resultado em 22 dias sem a necessidade de diálise após um ano. Conclusão: Devido ao diagnóstico precoce, o tratamento adequado foi instalado rapidamente proporcionando à paciente um aumento da expectativa e da qualidade de vida, evitando dependência de terapia renal substitutiva.Palavras-Chave: Granulomatose de Wegener, Plasmaferese, Doença renal crônica.  ABSTRACTIntroduction: The Wegener's Granulomatosis (WG) is a rare and idiopathic vasculitis associated with the presence of Antineutrophil Cytoplasmic Antibody (ANCA), that affects, preferentially, the small vessels. The clinical manifestations are diverse, occurring in over 90% of cases, symptoms in the respiratory tract. Kidney damage is a late and bad prognostic predictor. Morbidity in the medium and long term includes chronic renal failure. The probability of renal function maintenance success depends on serum creatinine concentration at the beginning of treatment that indicates the importance of early diagnosis and deployment of an appropriate therapy. Case Report: We present a case of a 61-year-old female patient, carrier of GW with advanced renal impairment presentation, not preceded by expected pulmonary symptoms. The immunosuppressive treatment associated with plasmapheresis allowed the improvement of the patient’s renal function, saving her from becoming dialysis-dependent Discussion: The patient developed the disease through asymptomatic renal failure, renal function with values that are compatible with the most advanced stage of chronic kidney disease, ultrasound of the kidneys without compatible changes and without the expected respiratory symptoms. According to the literature, the combination of immunosuppressive drugs and plasmapheresis is associated with renal recovery in three months with survival without dialysis for 12 months. In this case, a result was obtained in 22 days without the need for dialysis after one year. Conclusion: Due to the early diagnosis, appropriate treatment was quickly installed giving the patient increased life expectancy and quality, preventing dependence on renal replacement therapy.Keywords: Wegener’s granulomatosis, Plasmapheresis, Chronic renal failure.

scholarly journals Oral and Maxillo-Facial Manifestations of Systemic Diseases: An Overview

Medicina ◽  
2021 ◽  
Vol 57 (3) ◽  
pp. 271
Author(s):  
Saverio Capodiferro ◽  
Luisa Limongelli ◽  
Gianfranco Favia
Keyword(s):  
Early Diagnosis ◽  
Oral Cavity ◽  
Head And Neck ◽  
Clinical Outcomes ◽  
Systemic Disease ◽  
Clinical Manifestations ◽  
Systemic Diseases ◽  
Special Issue ◽  
Hard Tissues ◽  
Current Special Issue

Many systemic (infective, genetic, autoimmune, neoplastic) diseases may involve the oral cavity and, more generally, the soft and hard tissues of the head and neck as primary or secondary localization. Primary onset in the oral cavity of both pediatric and adult diseases usually represents a true challenge for clinicians; their precocious detection is often difficult and requires a wide knowledge but surely results in the early diagnosis and therapy onset with an overall better prognosis and clinical outcomes. In the current paper, as for the topic of the current Special Issue, the authors present an overview on the most frequent clinical manifestations at the oral and maxillo-facial district of systemic disease.

scholarly journals The Main Challenges in Systemic Lupus Erythematosus: Where Do We Stand?

2021 ◽  
Vol 10 (2) ◽  
pp. 243
Author(s):  
Matteo Piga ◽  
Laurent Arnaud
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Systemic Disease ◽  
Unmet Need ◽  
Clinical Manifestations ◽  
New Drugs ◽  
Systemic Lupus ◽  
Major Step ◽  
Damage Accrual

Systemic lupus erythematosus (SLE) is an immune-mediated multi-systemic disease characterized by a wide variability of clinical manifestations and a course frequently subject to unpredictable flares. Despite significant advances in the understanding of the pathophysiology and optimization of medical care, patients with SLE still have significant mortality and carry a risk of progressive organ damage accrual and reduced health-related quality of life. New tools allow earlier classification of SLE, whereas tailored early intervention and treatment strategies targeted to clinical remission or low disease activity could offer the opportunity to reduce damage, thus improving long-term outcomes. Nevertheless, the early diagnosis of SLE is still an unmet need for many patients. Further disentangling the SLE susceptibility and complex pathogenesis will allow to identify more accurate biomarkers and implement new ways to measure disease activity. This could represent a major step forward to find new trials modalities for developing new drugs, optimizing the use of currently available therapeutics and minimizing glucocorticoids. Preventing and treating comorbidities in SLE, improving the management of hard-to-treat manifestations including management of SLE during pregnancy are among the remaining major unmet needs. This review provides insights and a research agenda for the main challenges in SLE.

scholarly journals Defining Kawasaki disease and pediatric inflammatory multisystem syndrome-temporally associated to SARS-CoV-2 infection during SARS-CoV-2 epidemic in Italy: results from a national, multicenter survey

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Marco Cattalini ◽  
◽  
Sara Della Paolera ◽  
Fiammetta Zunica ◽  
Claudia Bracaglia ◽  
...  
Keyword(s):  
Kawasaki Disease ◽  
Clinical Laboratory ◽  
Troponin T ◽  
Inflammatory Diseases ◽  
Age At Onset ◽  
Laboratory Data ◽  
Clinical Manifestations ◽  
Chi Square ◽  
Pediatric Society ◽  
Multicenter Survey

Abstract Background There is mounting evidence on the existence of a Pediatric Inflammatory Multisystem Syndrome-temporally associated to SARS-CoV-2 infection (PIMS-TS), sharing similarities with Kawasaki Disease (KD). The main outcome of the study were to better characterize the clinical features and the treatment response of PIMS-TS and to explore its relationship with KD determining whether KD and PIMS are two distinct entities. Methods The Rheumatology Study Group of the Italian Pediatric Society launched a survey to enroll patients diagnosed with KD (Kawasaki Disease Group – KDG) or KD-like (Kawacovid Group - KCG) disease between February 1st 2020, and May 31st 2020. Demographic, clinical, laboratory data, treatment information, and patients’ outcome were collected in an online anonymized database (RedCAP®). Relationship between clinical presentation and SARS-CoV-2 infection was also taken into account. Moreover, clinical characteristics of KDG during SARS-CoV-2 epidemic (KDG-CoV2) were compared to Kawasaki Disease patients (KDG-Historical) seen in three different Italian tertiary pediatric hospitals (Institute for Maternal and Child Health, IRCCS “Burlo Garofolo”, Trieste; AOU Meyer, Florence; IRCCS Istituto Giannina Gaslini, Genoa) from January 1st 2000 to December 31st 2019. Chi square test or exact Fisher test and non-parametric Wilcoxon Mann-Whitney test were used to study differences between two groups. Results One-hundred-forty-nine cases were enrolled, (96 KDG and 53 KCG). KCG children were significantly older and presented more frequently from gastrointestinal and respiratory involvement. Cardiac involvement was more common in KCG, with 60,4% of patients with myocarditis. 37,8% of patients among KCG presented hypotension/non-cardiogenic shock. Coronary artery abnormalities (CAA) were more common in the KDG. The risk of ICU admission were higher in KCG. Lymphopenia, higher CRP levels, elevated ferritin and troponin-T characterized KCG. KDG received more frequently immunoglobulins (IVIG) and acetylsalicylic acid (ASA) (81,3% vs 66%; p = 0.04 and 71,9% vs 43,4%; p = 0.001 respectively) as KCG more often received glucocorticoids (56,6% vs 14,6%; p < 0.0001). SARS-CoV-2 assay more often resulted positive in KCG than in KDG (75,5% vs 20%; p < 0.0001). Short-term follow data showed minor complications. Comparing KDG with a KD-Historical Italian cohort (598 patients), no statistical difference was found in terms of clinical manifestations and laboratory data. Conclusion Our study suggests that SARS-CoV-2 infection might determine two distinct inflammatory diseases in children: KD and PIMS-TS. Older age at onset and clinical peculiarities like the occurrence of myocarditis characterize this multi-inflammatory syndrome. Our patients had an optimal response to treatments and a good outcome, with few complications and no deaths.

scholarly journals Prevalence and clinical phenotype of the triplicated α-globin genes and its ethnic and geographical distribution in Guizhou of China

2021 ◽  
Vol 14 (1) ◽  
Author(s):  
Xi Luo ◽  
Xiang-mei Zhang ◽  
Liu-song Wu ◽  
Jindong Chen ◽  
Yan Chen
Keyword(s):  
Genetic Counseling ◽  
Peripheral Blood ◽  
Clinical Phenotype ◽  
Globin Gene ◽  
Clinical Manifestations ◽  
Gene Mutations ◽  
Guizhou Province ◽  
Globin Genes ◽  
Phenotype Correlation ◽  
Chi Square

Abstract Background α-thalassemia is relatively endemic in Guizhou province of southwestern China. To predict the clinical manifestations of α-globin gene aberration for genetic counseling, we examined the prevalence of the α-globin triplication and the genotype–phenotype correlation in this subpopulation Methods A cohort of 7644 subjects was selected from nine ethnicities covering four regions in Guizhou province of China. Peripheral blood was collected from each participant for routine blood testing and hemoglobin electrophoresis. PCR-DNA sequencing and Gap-PCR were used to identify the thalassemia gene mutations. Chi-square tests and one-way analysis of variance (ANOVA) were used to statistically analyze the data. Results We found that the frequency of α-globin triplication in Guizhou province was 0.772% (59/7644). Genotypically, the αααanti4.2/αα accounted for 0.523% (40/7644), the αααanti3.7/αα for 0.235% (18/7644), and the αααanti3.7/–SEA for 0.013% (1/7644). The αααanti4.2/αα is more prevalent than the αααanti3.7/αα in Guizhou. In addition, the frequency of the HKαα/αα (that by GAP-PCR is like αααanti4.2/-α3.7) was 0.235% (18/7644). Ethnically, the Tujia group presented the highest prevalence (2.47%) of α-globin triplication. Geographically, the highest frequency of the α-globin triplication was identified in Qiannan region (2.23%). Of the triplicated α-globin cases, 5 coinherited with heterozygote β-thalassemia and presented various clinical manifestations of anemia. Conclusions These data will be used to update the Chinese triplicated α-globin thalassemia database and provide insights into the pathogenesis of thalassemia. These findings will be helpful for the diagnosis of thalassemia and future genetic counseling in those regions.

scholarly journals Bilateral Pleural Effusions as an Initial Presentation in Primary Sjögren’s Syndrome

2012 ◽  
Vol 2012 ◽  
pp. 1-3
Author(s):  
Go Makimoto ◽  
Michiko Asano ◽  
Nobukazu Fujimoto ◽  
Yasuko Fuchimoto ◽  
Katsuichiro Ono ◽  
...  
Keyword(s):  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Corticosteroid Treatment ◽  
Sjogren’S Syndrome ◽  
Pulmonary Involvement ◽  
Sjogren's Syndrome ◽  
Systemic Autoimmune Disease ◽  
Ophthalmological Examination ◽  
Pleural Effusions ◽  
Sjögren‘S Syndrome

Sjögren’s syndrome (SS) is a systemic autoimmune disease characterized by sicca symptoms. Interstitial pulmonary fibrosis and tracheobronchial sicca are the most common symptoms of pulmonary involvement in primary SjS, and they are rarely accompanied by serositis such as pleuritis or pericarditis. We report a case of SS presenting initially with bilateral pleural effusions. A 63-year old man was admitted to our hospital with a one-month history of cough, dyspnea, and right chest pain. Chest-computed tomography revealed bilateral pleural effusions. Serum anti-SS-A antibody titer was 1 : 256. Ophthalmological examination revealed a positive Schirmer test. Lip biopsy showed atrophy and plasmacytic infiltration of the salivary gland. Corticosteroid treatment was initiated. Pleural effusions were almost completely resolved by day 30. The patient has not experienced any recurrence.

Preeclampsia Part 2: fisiopatologia e trattamento

2019 ◽  
Vol 31 (2) ◽  
pp. 79-88
Author(s):  
Stefano Michelassi
Keyword(s):  
High Risk ◽  
Endothelial Dysfunction ◽  
Preterm Delivery ◽  
Systemic Inflammation ◽  
Systemic Disease ◽  
Clinical Manifestations ◽  
Morbidity And Mortality ◽  
Curative Treatment ◽  
Maternal Risks ◽  
New Onset

Preeclampsia is a pregnancy-specific disorder usually defined as new-onset hypertension and proteinuria after the 20th week of gestation. Preeclampsia is a systemic disease with multiorgan involvement, and it is associated with a high risk of maternal and fetal morbidity and mortality. To date its pathogenesis is not completely understood, but placental hypoxia or hypoxia/reoxigenation may be the basic condition leading to systemic inflammation and endothelial dysfunction that induce all the clinical manifestations of the disorder. Delivery is the only curative treatment. Indeed, for the management of preeclampsia one needs to consider both the maternal risks due to continued pregnancy and the fetal risks associated with induced preterm delivery.

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