Interstitial pneumonia with autoimmune features in a patient with melanoma differentiation-associated gene 5 (MDA5) antibody

2020 ◽  
Vol 13 (11) ◽  
pp. e234946
Author(s):  
Lih En Hong ◽  
Susanna Proudman ◽  
Vidya Limaye
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Serum Ferritin ◽  
Poor Prognosis ◽  
Serum Ferritin Level ◽  
Ferritin Level ◽  
High Serum ◽  
Amyopathic Dermatomyositis ◽  
Lung Infiltrates

Melanoma differentiation-associated gene 5 (MDA5) antibody, also known as anti-CADM140 antibody is recognised to be associated with rapidly progressive interstitial lung disease, which can be fatal within 3 months. It is also known to be associated with amyopathic dermatomyositis. We report a case of MDA5 antibody-associated interstitial pneumonia with autoimmune features, without cutaneous features of dermatomyositis, in a Sudanese patient with dual positive antibodies to Ro52. The patient notably had several features associated with poor prognosis, including age, high serum ferritin level, anti-Ro52 antibodies and progressive lung infiltrates during treatment.

PREDICTIVE SIGNIFICANCE OF HIGH SERUM FERRITIN LEVELS IN THE DEBUT OF MALIGNANT LYMPHOMA

2020 ◽  
pp. 74-81
Author(s):  
Babaeva T.N. ◽  
Seregina O.B. ◽  
Pospelova T.I.
Keyword(s):  
Serum Ferritin ◽  
Serum Ferritin Level ◽  
Ferritin Level ◽  
Prognostic Significance ◽  
Average Concentration ◽  
High Serum ◽  
Control Group ◽  
Malignant Neoplasms ◽  
Significant Prognostic Factor ◽  
Malignant Lymphomas

At present, the serum ferritin level is not included in the list of prognostic factors; however, it is known that its increased serum level in patients with malignant neoplasms relates with the tumor burden, the degree of disease activity and correlates with a worse prognosis in patients with hematologic malignancies.The normalization of serum ferritin level during remission period confirms the involving of hyperferritinemia in mechanisms of tumor progression and may testify for clinical importance of measurement of serum ferritin level in patients, including those with malignant lymphomas. Objective:The aim of this study was to assess of the prognostic significance of high ferritin levels at the onset of the disease in patients with malignant lymphomas. Materials and methods:98 patients with malignant lymphomaswere enrolled in this study, including 72 patients (73.5%) with non-Hodgkins lymphomas (NHL) and 26 patients (26.5%) with Hodgkin’s lymphoma (HL). The increased serum ferritin level (more than 350 ng/ml) was found in 53 (54.2%) patients with malignant lymphomas at the onset of disease and its average concentration was 587,62±131,6 ng/ml (8.3 times higher values of control group, p<0.001).Also the positive statistical correlationsbetween increased ferritin level and increased level of LDH (r=0.47, p<0.001, n=98) and C-reactive protein (r=0.41, p<0.001, n=98) as well as the presence of B-symptomswere found. The median OS was significantly shorter in the group of patients with increased ferritin level (more than 350 ng/ml) at the onset of disease in comparison with group of patients with normal ferritin level, where the median OS was not reach during the observation period. Patients with increased ferritin level before starting chemotherapy also showed worse results of overall survival and increased mortality risk (OR 8.122; 95% CI, 1.764-37.396;р<0.05) compare with a group of patients with ferritin level ˂350 hg/ml at the onset of disease. Conclusion:These results make it possible to include lymphomas’s patients with increased ferritin level at the onset of disease in the group with poor prognosis and lower OS, while the increased ferritin level in patients without previous blood transfusions should be considered as a significant prognostic factor.

PATTERN DYSTROPHY-LIKE CHANGES ASSOCIATED WITH VERY HIGH SERUM FERRITIN LEVEL IN β-THALASSEMIA MAJOR

2020 ◽  
Vol Publish Ahead of Print ◽  
Author(s):  
King Hans Kurnia ◽  
Elvioza Elvioza ◽  
Mohamad Sidik ◽  
Teny Tjitra Sari ◽  
Rita Sita Sitorus
Keyword(s):  
Serum Ferritin ◽  
Serum Ferritin Level ◽  
Ferritin Level ◽  
Thalassemia Major ◽  
High Serum ◽  
Pattern Dystrophy ◽  
Very High

scholarly journals Detection of endocrine disorders in young children with multi-transfused thalassemia major

2021 ◽  
Vol 47 (1) ◽  
Author(s):  
Ramadan A. Mahmoud ◽  
Ashraf Khodeary ◽  
Marwa S. Farhan
Keyword(s):  
Serum Ferritin ◽  
Serum Ferritin Level ◽  
Ferritin Level ◽  
Thalassemia Major ◽  
High Serum ◽  
Endocrine Disorders ◽  
Inclusion Criteria ◽  
Poor Patient ◽  
Poor Patient Compliance ◽  
Iron Chelating Agents

Abstract Background Beta thalassemia major (TM) is the most common inherited genetic disorder worldwide. Patients are at risk of iron overload, which leads to various forms of tissue damage, including endocrinopathies. The aim of this study was to evaluate the prevalence and risk factors of endocrine disorders in young patients with multi-transfused TM receiving iron chelation therapy. Methods The inclusion criteria included all known cases of TM according to hemoglobin electrophoresis data, aged 12 years or younger, during the study period. The patient’s age, gender, parent’s consanguinity, clinical examination, and types of iron chelating agents used were recorded. Serum ferritin level, complete blood count (CBC), blood glucose homeostasis, thyroid, and parathyroid functions were determined. Results One hundred twenty patients met the inclusion criteria; 70% of them had malnutrition. The presence of endocrine disorders was observed in 28/120 (23.33%) patients. The most common endocrine disorders were thyroid disorders, either subclinical or clinical hypothyroidism in 11/120 (9.17%) patients, followed by abnormalities in glucose homeostasis 9/120 (7.5%). The prevalence of impaired glucose tolerance, impaired fasting glucose, and diabetes mellitus in the present study was 5 (4.17%), 4 (3.33%), and 0 (00%), respectively, while the least frequent endocrine disorder seen in our patients was hypoparathyroidism in 8/120 (6.66%). We noted that high serum ferritin levels and poor patient compliance to therapy were significantly associated with increased endocrine disorders (OR 0.98, 95% CI 0.96–0.99, P = 0.003 and OR 0.38, 95% CI 0.16:0.93, P = 0.03, respectively). Combined chelating iron agents significantly decreased the prevalence of endocrine disorders when compared with monotherapy (OR 0.40, 95% CI 0.16:0.97, P = 0.04). Conclusion Endocrine disorders could occur in TM patients early before or equal to 12 years of life in about one-fourth of the patients. A high serum ferritin level and poor patient compliance to therapy were significantly associated with increased endocrine disorders. Combined iron-chelating agents were associated with a decreased prevalence of endocrine disorders when compared with monotherapy.

Bedeutung von Myositis-spezifischen Autoantikörpern bei der Dermatomyositis und Lungenbeteiligung

2021 ◽  
Vol 3 (1) ◽  
pp. 17-18
Author(s):  
Michael Sticherling
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Subcutaneous Emphysema ◽  
Poor Prognosis ◽  
Clinical Characteristics ◽  
Clinical Manifestations ◽  
Imaging Features ◽  
Amyopathic Dermatomyositis ◽  
Serological Tests ◽  
Medical University

<b>Objective:</b> To investigate the clinical characteristics of patients positive for anti-melanoma differentiation-associated gene 5 (MDA5) antibodies, and to analyse the potential pathogenesis of anti-MDA5 antibodies. <b>Methods:</b> The clinical manifestations, serological tests, imaging features, treatments, and prognoses of 32 anti-MDA5 antibody-positive patients diagnosed in the Rheumatology and Immunology Department of the Second Affiliated Hospital of Chongqing Medical University from September 2015 to August 2018 were analysed. <b>Results:</b> Of the 32 anti-MDA5 antibody-positive patients, eleven patients were clinically diagnosed with interstitial pneumonia with autoimmune features (IPAF), ten patients were diagnosed with clinically amyopathic dermatomyositis (CADM), six patients were diagnosed with dermatomyositis (DM) and five patients were diagnosed with anti-synthetase syndrome (ASS). Thirty patients had various degrees of pulmonary interstitial changes. The incidence of mortality, subcutaneous emphysema, hoarseness and dysphagia in patients who were positive for both anti-MDA5 and anti-Ro52 antibodies was significantly higher than in patients positive for only anti-MDA5 antibodies. The anti-MDA5 antibody-positive IPAF patients had a very poor prognosis, and mortality in these patients was as high as 54.55%. <b>Conclusion:</b> Anti-MDA5 antibodies are closely related to interstitial lung disease (ILD). The presence of both anti-MDA5 and anti-Ro52 antibodies indicates poor prognosis.

Relationship between high serum ferritin level and glaucoma in a South Korean population: the Kangbuk Samsung health study

2016 ◽  
Vol 100 (12) ◽  
pp. 1703-1707 ◽  
Author(s):  
Hyo Jung Gye ◽  
Joon Mo Kim ◽  
Chungkwon Yoo ◽  
Seong Hee Shim ◽  
Yu Sam Won ◽  
...  
Keyword(s):  
Serum Ferritin ◽  
Serum Ferritin Level ◽  
Ferritin Level ◽  
High Serum ◽  
Korean Population ◽  
Health Study ◽  
South Korean
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