scholarly journals ANTISYNTHETASE SYNDROME AND THE POSITION OF CLINICAL IMMUNOLOGIST

Author(s):  
Валентина Чоп’як ◽  
Христина Ліщук-Якимович ◽  
Роман Пукаляк ◽  
Омелян Синенький

Antisynthetase syndrome is a clinical and laboratory syndrome that develops in patients with idiopathic inflammatory myopathy and is characterized by the development of interstitial lung disease, namely fibrosing alveolitis syndrome, resistance to traditional corticoid therapy and the presence of myositis-specific antibodies.We present a clinical case of an antisynthetase syndrome in a middle-aged patient who has presented severe myalgic syndrome, photodermatosis, Raynaud’s phenomenon. The disease debuted with cutaneous (heliotropic erythema, erythematous rash on the skin of the upper torso) and myalgic symptoms, fever with next adding of the joint syndrome, as well as lung damage (pulmonitis and infiltrates). Immunological testing revealed anti-Jo-1, anti-PL-12-, anti-PL-7 antibodies («Polycheck», BIOCHECK, Germany). Since years, the patient has got the diagnosis of antisynthetase syndrome. The use of combined pulse therapy with cyclophosphamide and methylprednisolone, as well as the addition of high doses of vitamin D3 has contributed to the regression oflung damage and reduction of dermatomyositis activity.

2021 ◽  
Author(s):  
Mafalda Miranda Baleiras ◽  
Luís Maduro ◽  
Carolina Vasques ◽  
Filipa Ferreira ◽  
Marta Mesquita Pinto ◽  
...  

Prostate cancer is the second most frequent malignancy in men worldwide and the fifth leading cause of death. Dermatomyositis (DM) is a rare idiopathic inflammatory myopathy characterized by musculocutaneous manifestations. However, DM can also present as a paraneoplastic syndrome of an underlying neoplasm. We report a case of a 65-year-old man diagnosed with prostate adenocarcinoma in the setting of severe dysphagia, muscle weakness and a facial erythematous rash. At first, the DM-related symptoms resolved with the initial treatment for the underlying malignancy. Yet, they flared up as the tumor progressed. To sum up, DM is a rare systemic disorder with unknown etiology. There is a well-established association between DM and malignancy. Malignancy-headed therapy can improve DM manifestations and the recurrence of DM symptoms may act as an early warning of malignancy relapse.


2018 ◽  
Vol 43 (12) ◽  
pp. 939-940
Author(s):  
Paula Fernández-Rodríguez ◽  
Juan José Martín-Marcuartu ◽  
Jose Manuel Jiménez-Hoyuela

2021 ◽  
pp. 230-237
Author(s):  
V. V. Salukhov ◽  
N. I. Gulyaev ◽  
E. V. Dorokhina

The mechanisms of COVID-19-associated coagulopathy (CAC) are complex and differ in many ways from the standard mechanisms of thrombosis in critically ill patients. This review presents the pathogenesis, diagnosis, and comparison of various types of coagulopathy with SAS. During COVID-19 infection, the number of sudden deaths outside the hospital increased. One possible reason is the high incidence of serious thrombotic events in patients with COVID-19. However, the pathogenesis of these life-threatening events is multifactorial and requires independent discussion.Deviations in laboratory studies of the hemostatic system in patients infected with SARS-CoV-2 with a severe course indicate the activation of the blood coagulation system corresponding to sepsis-induced coagulopathy (SIC) or DIC. However, hemostasis disorders in COVID-19 have characteristics that distinguish them from DIC in sepsis.The clinical and laboratory features of CAC overlap with hemophagocytic syndrome, antiphospholipid syndrome, and thrombotic microangiopathy. The review presents data on their similarities and differences.Inadequate diagnosis or inadequate treatment of hypercoagulability may explain the high incidence of unexplained deaths from COVID-19. They can be associated with potentially preventable microvascular and macrovascular thrombosis and subsequent cardiovascular complications, including myocardial injury and infarction, as well as insufficient information content of biomarkers for their assessment.Research to identify the most informative biomarkers for decision-making to intensify anticoagulant prophylaxis in patients with severe COVID-19 is progressing rapidly, with increasing focus on TEG and ROTEM.The review presents changes in CAC during hormone therapy for COVID-19-associated lung damage. Pulse therapy with high doses of GCS has a rapid anti-inflammatory effect, but at the same time increases the level of D-dimer, which increases the risk of venous thrombosis and thromboembolism. 


2019 ◽  
Vol 1 (9) ◽  
pp. 53-57
Author(s):  
T. N. Gavva ◽  
L. V. Kuzmenkova ◽  
Yu. N. Fedulaev ◽  
T. V. Pinchuk ◽  
D. D. Kaminer ◽  
...  

A case of lung damage in systemic lupus erythematosus (SLE) in a 33-year-old woman is described. This case is of clinical interest due to the complexity of diagnosis due to the fact that SLE is a disease with diverse clinical manifestations involving many organs and systems, which often makes it difficult to timely recognize the onset of the disease. SLE still remains a challenge and requires special attention to the patient s history, clinical and laboratory parameters of the patient, as well as specific immunological examinations.


2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
E Bollache ◽  
AT Huber ◽  
J Lamy ◽  
E Afari ◽  
TM Bacoyannis ◽  
...  

Abstract Funding Acknowledgements Type of funding sources: None. Background. Recent studies revealed the ability of MRI T1 mapping to characterize myocardial involvement in both idiopathic inflammatory myopathy (IIM) and acute viral myocarditis (AVM), as compared to healthy controls. However, neither myocardial T1 nor T2 maps were able to discriminate between IIM and AVM patients, when considering conventional myocardial mean values and derived indices such as lambda and extracellular volume. Purpose. To investigate the ability of T1 mapping-derived texture analysis to differentiate IIM from AVM. Methods. Forty patients, 20 with IIM (51 ± 17 years, 9 men) and 20 with AVM (34 ± 13 years, 16 men) underwent 1.5T MRI T1 mapping using a modified Look-Locker inversion-recovery sequence before and 15 minutes after injection of a gadolinium contrast agent. After manual delineation of endocardial and epicardial borders and co-registration of all inversion time images, native and post-contrast T1 maps were estimated. Myocardial texture analysis was performed on native T1 maps. Textural features such as: autocorrelation, contrast, dissimilarity, energy and sum entropy were used to build a least squares-based linear regression model. Finally, receiver operating characteristic (ROC) analysis was used to investigate the ability of such texture features score to classify IIM vs. AVM patients, compared to the performance of mean myocardial T1. A Wilcoxon rank-sum test was also used to test difference significance between groups. Results. Both native and post-contrast mean myocardial T1 values were comparable between IIM (native: 1022 ± 43 ms; post-contrast: 319 ± 44 ms) and AVM (1056 ± 59 ms, p = 0.07; 318 ± 35 ms, p = 0.90, respectively) groups. Results of ROC analyses are provided in the Table, indicating that a better discrimination between IIM and AVM patients was obtained when using texture features, with higher AUC and accuracy than mean T1 values (Figure). Conclusion. Texture analysis derived from MRI T1 maps without contrast agent injection was able to discriminate between IIM and AVM with higher accuracy, sensitivity and specificity than conventional T1 indices. Such analysis could provide a useful myocardial signature to help diagnose and manage cardiac alterations associated with IIM in patients presenting with myocarditis and primarily suspected of AVM. Table Area under curve (AUC) Accuracy Sensitivity Specificity Native T1 0.67 0.70 0.65 0.75 Post-contrast T1 0.49 0.60 0.25 0.95 Texture features score 0.85 0.82 0.90 0.75 ROC analyses for classification between IIM and AVM patients Abstract Figure


Author(s):  
Tatiana Cobo-Ibáñez ◽  
Carlos Sánchez-Piedra ◽  
Laura Nuño-Nuño ◽  
Iván Castellví ◽  
Irene Carrión-Barberà ◽  
...  

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