THU0286 The Efficacy and The Long-Term Prognosis of Rituximab for Refractory Thrombotic Microangiopathy Associated with Connective Tissue Diseases

2016 ◽  
Vol 75 (Suppl 2) ◽  
pp. 290.2-290
Author(s):  
A. Kondo ◽  
H. Dobashi ◽  
Y. Takeuchi ◽  
T. Kameda ◽  
M. Izumikawa ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Thrombotic Microangiopathy ◽  
Connective Tissue Diseases ◽  
Long Term Prognosis

scholarly journals Long-term iloprost infusion therapy for severe pulmonary hypertension in patients with connective tissue diseases

1994 ◽  
Vol 37 (10) ◽  
pp. 1528-1533 ◽  
Author(s):  
Jose De La Mata ◽  
Miguel A. Gomez-Sanchez ◽  
Mercedes Aranzana ◽  
Juan J. Gomez-Reino
Keyword(s):  
Pulmonary Hypertension ◽  
Connective Tissue ◽  
Connective Tissue Diseases ◽  
Infusion Therapy ◽  
Severe Pulmonary Hypertension ◽  
Iloprost Infusion

scholarly journals Photodermatoses in Childhood

2021 ◽  
Vol 20 (5) ◽  
pp. 360-369
Author(s):  
Nikolay N. Murashkin ◽  
Eduard T. Ambarchian ◽  
Roman V. Epishev ◽  
Alexander I. Materikin ◽  
Leonid A. Opryatin ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Clinical Symptom ◽  
Metabolic Disorders ◽  
Connective Tissue Diseases ◽  
Heterogeneous Group ◽  
Diverse Group ◽  
Skin Hypersensitivity ◽  
Timely Diagnosis ◽  
Laboratory Features

Photodermatoses is a heterogeneous group of diseases resulting from abnormal skin hypersensitivity to sunlight and presented as local or generalized rashes. Specific sensitivity of children's skin to ultraviolet is often the first sign or clinical symptom of photodermatosis. Abnormal photosensitivity can be represented by diverse group of primary idiopathic conditions or photo-mediated aggravation of existing dermatosis. Number of genetic genodermatoses, metabolic disorders and connective tissue diseases is also widely known. These conditions can manifest with photosensitivity associated to other extracutaneous clinical and laboratory features. Timely diagnosis of photosensitivity in childhood allows to minimize long-term complications associated with insufficient photoprotection.

scholarly journals A clinical case of long-term differential diagnosis of a disseminated process in the lungs as a manifestation of mixed connective tissue disease

2020 ◽  
Vol 43 (4) ◽  
pp. 509-521
Author(s):  
Elina N. Solovyova ◽  
◽  
Tatyana Yu. Kalyuta ◽  
Oleg A. Kazhekin ◽  
Nadezhda A. Glukhova ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Lupus Erythematosus ◽  
Clinical Case ◽  
Connective Tissue Diseases ◽  
Lung Damage ◽  
Common Causes ◽  
Long Time

Connective tissue diseases (CTD) are common causes of disseminated small – focal lung damage. The complexity of diagnostic approach in such cases occur due to intolerance of many symptoms and syndromes, that can also relate to diseases of other different etiologies. It is often not possible to identify the pathognomonic syndrome in CTD, as well as to establish an accurate diagnosis after a long time. This complicates the selection of effective treatment and, accordingly, the inability to predict the course and development of the disease. Sometimes, due to the intersection of symptoms, the diagnosis sounds like «MCTD» (mixed connective tissue diseases) – a rare systemic connective tissue disease, characterized by a combination of individual signs of systemic lupus erythematosus, systemic scleroderma, rheumatoid arthritis, polymyositis. This publication presents a clinical case of a long-term course of mixed MCTD in a patient who has been observed for more than 10 years in different hospitals, and by outpatient observation, and within this year’s differential diagnosis with other CTD and infectious diseases was held, including rheumatic fever.

scholarly journals Pectus excavatum and heritable disorders of the connective tissue

2013 ◽  
Vol 5 (3) ◽  
pp. 15 ◽  
Author(s):  
Francesca Tocchioni ◽  
Marco Ghionzoli ◽  
Antonio Messineo ◽  
Paolo Romagnoli
Keyword(s):  
Mitral Valve ◽  
Connective Tissue ◽  
Pectus Excavatum ◽  
Connective Tissue Disorder ◽  
Vascular Involvement ◽  
Connective Tissue Disorders ◽  
Chest Wall Deformity ◽  
Long Term Prognosis ◽  
Heritable Connective Tissue Disorders

<em>Pectus excavatum</em>, the most frequent congenital chest wall deformity, may be rarely observed as a sole deformity or as a sign of an underlying connective tissue disorder. To date, only few studies have described correlations between this deformity and heritable connective tissue disorders such as Marfan, Ehlers-Danlos, Poland, MASS (Mitral valve prolapse, not progressive Aortic enlargement, Skeletal and Skin alterations) phenotype among others. When concurring with connective tissue disorder, cardiopulmonary and vascular involvement may be associated to the thoracic defect. Ruling out the concomitance of <em>pectus excavatum</em> and connective tissue disorders, therefore, may have a direct implication both on surgical outcome and long term prognosis. In this review we focused on biological bases of connective tissue disorders which may be relevant to the pathogenesis of <em>pectus excavatum</em>, portraying surgical and clinical implication of their concurrence.

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