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1.
Article in Chinese | WPRIM | ID: wpr-878714

ABSTRACT

Objective To investigate the clinical features of neuromyelitis optica spectrum disorders(NMOSD)with connective tissue diseases(CTD). Methods Clinical data of 16 NMOSD-CTD patients and 54 NMOSD patients admitted to the Second Affiliated Hospital of Fujian Medical University from January 2015 to February 2020 were collected.The initial symptom,intracranial lesion,spinal cord lesion,laboratory examination and treatment response were compared between the two groups. Results The incidence of Sjögren's syndrome(SS)was the highest(10/16,62.5%)in NMOSD-CTD group.The NMOSD-CTD group had significantly higher positive rate of aquaporin-4 immunoglobulin G(AQP4-IgG)in serum or cerebrospinal fluid(100% vs. 70.2%,P=0.009),higher positive rates of serum anti-nuclear antibodies,anti Sjögren's syndrome A antibodies and anti-Ro52 autoantibodies(P0.05).Conclusions NMOSD is often complicated with CTD,and SS is the most common one.The positive rate of serum or cerebrospinal AQP4-IgG and the seropositivity of several other autoantibodies in NMOSD-CTD patients were higher than those in NMOSD patients.Neurological impairment in NMOSD-CTD patients were severer,which should arouse attention of clinicians.


Subject(s)
Aquaporin 4 , Autoantibodies , Connective Tissue Diseases/epidemiology , Humans , Immunoglobulin G , Neuromyelitis Optica/epidemiology
2.
Article in English | LILACS, BBO | ID: biblio-1287497

ABSTRACT

ABSTRACT Objective: To analyze periodontal comparison between Systemic Lupus Erythematosus (SLE) subject and healthy control. Material and Methods: This descriptive cross-sectional study included 122 subjects, 61 SLE patients and 61 healthy subjects who visited the Rheumatology Department, Dr. Saiful Anwar General Hospital, Malang, during 2017-2018. Clinical examination of SLE was using Mexican SLE Disease Activity Index and oral cavity conditions were assessed using the periodontal index, gingival index, calculus index, bleeding on probing, clinical attachment loss and mobility teeth. Results: The age of SLE patients ranged from 18-55 years old with the mean age of 29.50 ± 9.57 years old. Periodontitis was higher in SLE patients (88.5%) than healthy subjects (22.95%). In addition, periodontitis occurrence in SLE (2.66 ± 1.02) was significantly different (p<0.001) compared to healthy subjects (0.51 ± 0.81). Conclusion: This study found higher rates of periodontitis, gingivitis, bleeding on probing, clinical attachment loss, and mobility tooth among SLE patients compared to healthy subjects. Periodontitis was also found to be higher along with more severe SLE group.


Subject(s)
Humans , Male , Female , Adult , Middle Aged , Periodontal Diseases/pathology , Tooth Mobility , Periodontal Index , Connective Tissue Diseases , Lupus Erythematosus, Systemic/pathology , Periodontitis , Clinical Diagnosis , Dental Plaque Index , Oral Health , Epidemiology, Descriptive , Cross-Sectional Studies/methods , Data Interpretation, Statistical , Statistics, Nonparametric , Gingivitis , Indonesia/epidemiology
3.
Rev. argent. reumatolg. (En línea) ; 31(2): 25-30, jun. 2020. tab
Article in Spanish | LILACS, BINACIS | ID: biblio-1143928

ABSTRACT

Objetivos: Determinar la relación de los anticuerpos con los antígenos del núcleo extraíble y las enfermedades del tejido conectivo identificadas por Immunoblot en un hospital de Lima, Perú. Material y métodos: Estudio de tipo observacional, ciencias básicas, analíticas y transversales, realizado en el Servicio de Inmunología del Hospital Nacional Arzobispo Loayza entre enero de 2018 y junio de 2018. Analizamos 291 historias clínicas de pacientes con enfermedad del tejido conectivo y para la detección de anticuerpos contra los antígenos extraíbles del núcleo se empleó el método de Immunoblots. Resultados: La frecuencia de los anticuerpos contra antígenos nucleares extraíbles en pacientes con enfermedad del tejido conectivo identificados por Immunoblot fue 789 (100%). Se demostró que existe una relación significativa p <0.05 de Anti-histonas (X2 = 64.19; p = 0,000), anti-nucleosomas (X2 = 71,16; p = 0,000), anti-dsDNA (X2 = 71,44; p = 0,000), anti-SM (X2 = 10,08; p = 0,003) y lupus eritematoso sistémico con prueba de Chi-cuadrado de Pearson. Se demostró que existe una relación significativa p <0.05 del Anti-SSA (X2 = 61,33; p = 0.001), anti-SSB (x2 = 51,00; p = 0.001), anti-Ro 52 (X2 = 62,60; p = 0,000) y síndrome de Sjogren con prueba de Chi-cuadrado de Pearson. Se demostró que existe una relación significativa p <0.05 de Anti-CENP B (p = 0.001) y calcinosis, fenómeno de Raynaud, dismotilidad esofágica, esclerodactilia y Telangiectasia (CREST) con Fisher. Conclusiones: Existe relación de anticuerpos con antígenos de núcleo extraíbles y lupus eritematoso sistémico, síndrome de Sjogren, enfermedad mixta del tejido conectivo, enfermedad del CREST, esclerodermia y polimiositis.


Objectives: To determine the relationship of antibodies to extractable nucleus antigens and connective tissue diseases identified by Immunoblot in a hospital in Lima, Peru. Material and methods: Study of the observational type, basic sciences, analytical and transversal, carried out in the Immunology service of the national Hospital Archbishop Loayza between January 2018 and June 2018. We analyzed 291 clinical histories of patients with connective tissue disease and for the detection of antibodies to the extractable antigens of the nucleus the method of Immunoblot was employed. Results: The frequency of the antibodies against extractable nuclear antigens in patients with connective tissue disease identified by Immunoblot was 789 (100%). It was demonstrated that there is significant relationship p < 0.05 of Anti-histones (X2 = 64.19; p = 0,000), anti-nucleosomas (X2 = 71,16; p = 0,000), anti-dsDNA (X2 = 71,44; p = 0,000), anti-SM (X2 = 10,08; p = 0,003) and Lupus Systemic erythematosus with Pearson Chi-square test. It was demonstrated that there is significant relationship p < 0.05 of the Anti-SSA (X2 = 61,33; p = 0.001), anti-SSB (X2 = 51,00; p = 0.001), anti-Ro 52 (X2 = 62,60; p = 0,000) and Sjogren's syndrome with Pearson Chi-square test. It was demonstrated that there is significant relationship p < 0.05 of Anti-CENP B (p = 0.001) and calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly and Telangiectasia (CREST) with exact Fisher statistician. Conclusions: There is a relationship of antibodies to extractable nucleus antigens and systemic lupus erythematosus, Sjogren's syndrome, mixed connective tissue disease, calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly and Telangiectasias (CREST), Scleroderma and Polymyositis.


Subject(s)
Humans , Antibodies , Connective Tissue , Connective Tissue Diseases , Mixed Connective Tissue Disease , Antigens
4.
Article in English | WPRIM | ID: wpr-811266

ABSTRACT

Osteogenesis imperfecta is a heterogeneous group of connective tissue diseases that is predominantly characterized by bone fragility and skeletal deformity. Two siblings with undiagnosed type I osteogenesis imperfecta underwent orthognathic surgery for the treatment of facial asymmetry and mandibular prognathism. The authors report two cases of combined orthodontics and orthognathic surgery in patients with type I osteogenesis imperfecta, mandibular prognathism, and facial asymmetry.


Subject(s)
Congenital Abnormalities , Connective Tissue Diseases , Facial Asymmetry , Humans , Orthodontics , Orthognathic Surgery , Osteogenesis Imperfecta , Osteogenesis , Prognathism , Siblings
5.
Rev. chil. enferm. respir ; 35(4): 266-267, dic. 2019.
Article in Spanish | LILACS | ID: biblio-1092704

ABSTRACT

Para el diagnóstico certero de fibrosis pulmonar idiopática (FPI) es de vital importancia la presencia de un patrón tomográfico definitivo de neumonía intersticial usual (NIU), en un contexto clínico adecuado. El interrogatorio dirigido, el uso de cuestionarios validados, una evaluación reumatológica acuciosa y exámenes complementarios son importantes para descartar causas secundarias de fibrosis pulmonar como neumonitis por hipersensibilidad (NHS), enfermedades del tejido conectivo (ETC), toxicidad por drogas y algunas neumoconiosis que pueden imitar el patrón radiológico y muchas veces dificultar un diagnóstico adecuado de FPI.


For the accurate diagnosis of idiopathic pulmonary fibrosis (IPF), the presence of a definitive tomographic pattern of usual interstitial pneumonia (UIP) is of vital importance, in an appropriate clinical context. Targeted interrogation, the use of valid questionnaires, an acute rheumatologic evaluation and complementary examinations are important to rule out secondary causes such as hypersensitivity pneumonitis (HP), connective tissue diseases (CTD), drug toxicity and some pneumoconiosis that can mimic the radiological pattern and often hinder a clear diagnosis of IPF.


Subject(s)
Humans , Idiopathic Pulmonary Fibrosis/diagnosis , Pneumoconiosis/diagnosis , Connective Tissue Diseases/diagnosis , Diagnosis, Differential , Alveolitis, Extrinsic Allergic/diagnosis
6.
Rev. chil. enferm. respir ; 35(4): 278-281, dic. 2019. tab
Article in Spanish | LILACS | ID: biblio-1092707

ABSTRACT

Las Enfermedades del Tejido Conectivo (ETC) comprenden un grupo heterogéneo de patologías multisistémicas de origen autoinmune. La Enfermedad pulmonar intersticial (EPI) asociada a ETC (EPI-ETC) es frecuente y empeora el pronóstico de la ETC. Las EPI-ETC representan aproximadamente 15-30% del total las EPI y se presentan con las mismas formas histopatológicas y radiológicas descritas para las EPI idiopáticas. Esto pone en evidencia la importancia de incorporar en forma rutinaria a reumatología en el comité multidisciplinario para el diagnóstico y manejo de las EPI.


Connective Tissue Diseases (CTD) comprise a heterogeneous group of multisystemic pathologies of autoimmune origin. Interstitial lung disease (ILD) associated with CTD (CTD-ILD) is common and and it worsens the prognosis of CTD. CTD-ILD represent approximately 15-30% of the universe of ILD and have the same histopathological and radiological forms described for idiopathic ILD. This highlights the importance of routinely incorporate a rheumatologist into the multidisciplinary committee for the diagnosis and management of ILD.


Subject(s)
Humans , Rheumatic Diseases/complications , Connective Tissue Diseases/complications , Idiopathic Pulmonary Fibrosis/diagnosis , Idiopathic Pulmonary Fibrosis/immunology , Rheumatic Diseases/diagnosis , Connective Tissue Diseases/diagnosis
7.
Rev. méd. Chile ; 147(11): 1458-1467, nov. 2019. tab
Article in Spanish | LILACS | ID: biblio-1094176

ABSTRACT

Interstitial lung diseases are a broad, diverse, challenging group of diseases, most of them chronic whose prognosis is not good. In the last two decades there have been considerable advances in the knowledge of the epidemiology, pathological and genetic bases and treatment of several of these diseases. This article summarizes and presents updated information about their classification, new knowledge on genetics and treatments in idiopathic pulmonary fibrosis, advances in the diagnosis and management of hypersensitivity pneumonitis and a review of the broad spectrum of interstitial diseases associated with connective tissue diseases. Several clinical trials are currently underway whose results will be available in the coming years and will provide more information and tools to improve the treatment of these patients.


Subject(s)
Lung Diseases, Interstitial/classification , Lung Diseases, Interstitial/diagnosis , Prognosis , Connective Tissue Diseases/diagnosis , Diagnosis, Differential , Idiopathic Pulmonary Fibrosis/diagnosis , Alveolitis, Extrinsic Allergic/diagnosis
8.
Article in English | WPRIM | ID: wpr-785302

ABSTRACT

Connective tissue diseases (CTDs) can affect all compartments of the lungs, including airways, alveoli, interstitium, vessels, and pleura. CTD-associated lung diseases (CTD-LDs) may present as diffuse lung disease or as focal lesions, and there is significant heterogeneity between the individual CTDs in their clinical and pathological manifestations. CTD-LDs may presage the clinical diagnosis a primary CTD, or it may develop in the context of an established CTD diagnosis. CTD-LDs reveal acute, chronic or mixed pattern of lung and pleural manifestations. Histopathological findings of diverse morphological changes can be present in CTD-LDs airway lesions (chronic bronchitis/bronchiolitis, follicular bronchiolitis, etc.), interstitial lung diseases (nonspecific interstitial pneumonia/fibrosis, usual interstitial pneumonia, lymphocytic interstitial pneumonia, diffuse alveolar damage, and organizing pneumonia), pleural changes (acute fibrinous or chronic fibrous pleuritis), and vascular changes (vasculitis, capillaritis, pulmonary hemorrhage, etc.). CTD patients can be exposed to various infectious diseases when taking immunosuppressive drugs. Histopathological patterns of CTD-LDs are generally nonspecific, and other diseases that can cause similar lesions in the lungs must be considered before the diagnosis of CTD-LDs. A multidisciplinary team involving pathologists, clinicians, and radiologists can adequately make a proper diagnosis of CTD-LDs.


Subject(s)
Bronchiolitis , Communicable Diseases , Connective Tissue Diseases , Connective Tissue , Diagnosis , Fibrin , Hemorrhage , Humans , Idiopathic Pulmonary Fibrosis , Lung Diseases , Lung Diseases, Interstitial , Lung , Pleura , Pleural Diseases , Population Characteristics
9.
Article in Chinese | WPRIM | ID: wpr-813071

ABSTRACT

To investigate the clinical characteristics and prognosis for connective tissue disease (CTD) with cryptococcal meningitis (CM). 
 Methods: Clinical data of 18 patients with CTD complicated with cryptococcal meningitis diagnosed by Rheumatology and Immunology Department, Xiangya Hospital, Central South University from January 2000 to January 2017, were retrospectively analyzed.
 Results: The common symptoms of CTD patients with CM were headache, fever, nausea, and vomiting. Patients with severe clinical manifestations, such as convulsions and disturbance of consciousness, all died. Logistic regression analysis showed that disturbance of consciousness and decreased peripheral blood lymphocyte count might be the related factors of poor prognosis of CTD patients with CM (P<0.05). The mortality rate of CTD with CM was 61.11%, and the effective rate of treatment for this disease was 38.89%.
 Conclusion: CTD patients with cryptococcal meningitis have a high risk of death. Severe clinical symptoms, such as disturbance of consciousness and lower peripheral blood lymphocyte count, are associated with its poor prognosis.


Subject(s)
Connective Tissue Diseases , Fever , Humans , Meningitis, Cryptococcal , Retrospective Studies , Vomiting
10.
Article in English | WPRIM | ID: wpr-766188

ABSTRACT

Overlap syndrome is defined as a disease entity that satisfies the classification criteria of at least two connective tissue diseases occurring concurrently or separately in a single patient. Here, we report a rare case of a 59-year-old woman with diffuse systemic sclerosis with lung involvement-rheumatoid arthritis overlap syndrome accompanied by cutaneous sarcoidosis. Although there is no consensus for the optimal treatment of overlap syndrome to date, this case of co-existing rheumatoid arthritis and systemic sclerosis with interstitial lung disease successfully responded to abatacept.


Subject(s)
Abatacept , Arthritis , Arthritis, Rheumatoid , Classification , Connective Tissue Diseases , Consensus , Female , Humans , Lung , Lung Diseases, Interstitial , Middle Aged , Sarcoidosis , Scleroderma, Diffuse , Scleroderma, Systemic
11.
Article in English | WPRIM | ID: wpr-762748

ABSTRACT

Ehlers-Danlos syndrome (EDS) is an inherited disorder of collagen biosynthesis and structure, characterized by skin hyperextensibility, joint hypermobility, aberrant scars, and tissue friability. Besides the skin, skeleton (joint) and vessels, other organs such as the eyes and the intestine can be affected in this syndrome. Accordingly, interdisciplinary cooperation is necessary for a successful treatment. Three basic surgical problems are arising due to an EDS: decreased the strength of the tissue causes making the wound dehiscence, increased bleeding tendency due to the blood vessel fragility, and delayed wound healing period. Surgery patients with an EDS require an experienced surgeon in treating EDS patients; the treatment process requires careful tissue handling and a long postoperative care. A surgeon should also recognize whether the patient shows a resistance to local anesthetics and a high risk of hematoma formation. This report describes a patient with a wide open wound on the foot dorsum and delayed wound healing after the primary approximation of the wound margins.


Subject(s)
Anesthetics, Local , Blood Vessels , Cicatrix , Collagen , Connective Tissue Diseases , Ehlers-Danlos Syndrome , Foot , Hematoma , Hemorrhage , Humans , Intestines , Joint Instability , Postoperative Care , Skeleton , Skin , Wound Healing , Wounds and Injuries
12.
Article in English | WPRIM | ID: wpr-761966

ABSTRACT

Idiopathic interstitial pneumonia (IIP) is a histologically identifiable pulmonary disease without a known cause that usually infiltrates the lung interstitium. IIP is largely classified into idiopathic pulmonary fibrosis, idiopathic non-specific interstitial pneumonia, respiratory bronchiolitis-interstitial lung disease (ILD), cryptogenic organizing pneumonia, desquamative interstitial pneumonia, and acute interstitial pneumonia. Each of these diseases has a different prognosis and requires specific treatment, and a multidisciplinary approach that combines chest high-resolution computed tomography (HRCT), histological findings, and clinical findings is necessary for their diagnosis. Diagnosis of IIP is made based on clinical presentation, chest HRCT findings, results of pulmonary function tests, and histological findings. For histological diagnosis, video-assisted thoracoscopic biopsy and transbronchial lung biopsy are used. In order to identify ILD associated with connective tissue disease, autoimmune antibody tests may also be necessary. Many biomarkers associated with disease prognosis have been recently discovered, and future research on their clinical significance is necessary. The diagnosis of ILD is difficult because patterns of ILD are both complicated and variable. Therefore, as with other diseases, accurate history taking and meticulous physical examination are crucial.


Subject(s)
Biomarkers , Biopsy , Classification , Connective Tissue Diseases , Cryptogenic Organizing Pneumonia , Diagnosis , Idiopathic Interstitial Pneumonias , Idiopathic Pulmonary Fibrosis , Lung , Lung Diseases , Lung Diseases, Interstitial , Physical Examination , Prognosis , Respiratory Function Tests , Thorax
13.
Article in English | WPRIM | ID: wpr-761964

ABSTRACT

Connective tissue disease (CTD) is a collection of disorders characterized by various signs and symptoms such as circulation of autoantibodies in the entire system causing damage to internal organs. Interstitial lung disease (ILD) which is associated with CTD is referred to as CTD-ILD. Patients diagnosed with ILD should be thoroughly examined for the co-occurrence of CTD, since the treatment procedures and prognosis of CTD-ILD are vary from those of idiopathic interstitial pneumonia. The representative types of CTD which may accompany ILD include rheumatoid arthritis, systemic sclerosis (SSc), Sjögren's syndrome, mixed CTD, idiopathic inflammatory myopathies, and systemic lupus erythematous. Of these, ILD most frequently co-exists with SSc. If an ILD is observed in the chest, high resolution computed tomography and specific diagnostic criteria for any type of CTD are met, then a diagnosis of CTD-ILD is made. It is challenging to conduct a properly designed randomized study on CTD-ILD, due to low incidence. Therefore, CTD-ILD treatment approach is yet to been established in absence of randomized controlled clinical trials, with the exception of SSc-ILD. When a patient is presented with acute CTD-ILD or if symptoms occur due to progression of the disease, steroid and immunosuppressive therapy are generally considered.


Subject(s)
Arthritis, Rheumatoid , Asian Continental Ancestry Group , Autoantibodies , Connective Tissue Diseases , Connective Tissue , Diagnosis , Disease Management , Guidelines as Topic , Humans , Idiopathic Interstitial Pneumonias , Incidence , Lung , Lung Diseases, Interstitial , Myositis , Prognosis , Scleroderma, Systemic , Thorax
15.
Korean Journal of Medicine ; : 173-181, 2019.
Article in Korean | WPRIM | ID: wpr-741136

ABSTRACT

Idiopathic pulmonary fibrosis (IPF) is a progressive fibrosing interstitial lung disease characterized by worsening lung function and dyspnea. The prognosis of IPF patients is poor, as median survival is approximately 3 years. However, recently developed IPF-specific therapies have shown improved efficacies in terms of reducing lung function decline and mortality. Therefore, the early recognition and accurate diagnosis of IPF are crucial. In 2018, new guidelines for the diagnosis of IPF were published by the Fleischner Society and by the American Thoracic Society/European Respiratory Society/Japanese Respiratory Society/Latin American Thoracic Society (ATS/ERS/JRS/ALAT). Both guidelines emphasize the need for a thorough history taking and physical examination to exclude an alternative diagnosis, such as exposure-related or connective tissue disease. The most informative initial examination is high-resolution computed tomography, the results of which can indicate the need for bronchoalveolar lavage or surgical lung biopsy, based on a multidisciplinary discussion of the findings and the patient's clinical condition. A multidisciplinary discussion of the clinico-radiologic-pathologic findings is currently the gold standard in the diagnoisis of IPF and will allow the more effective and timely treatment of these patients.


Subject(s)
Biopsy , Bronchoalveolar Lavage , Connective Tissue Diseases , Diagnosis , Dyspnea , Humans , Idiopathic Pulmonary Fibrosis , Lung , Lung Diseases, Interstitial , Mortality , Physical Examination , Prognosis
16.
Korean Circulation Journal ; : 353-360, 2019.
Article in English | WPRIM | ID: wpr-738786

ABSTRACT

BACKGROUND AND OBJECTIVES: Udenafil, a new phosphodiesterase-5 inhibitor (PDE5i), has been used to treat erectile dysfunction. Given the proven benefit of PDE5i in pulmonary arterial hypertension (PAH), we evaluated serial hemodynamic changes after single udenafil administration to determine the appropriate therapeutic dose. METHODS: Eighteen patients were randomly allocated into one of 3 groups: placebo, udenafil 50 mg (U50), and udenafil 100 mg (U100). Diagnosis for inclusion was idiopathic PAH or PAH associated with connective tissue disease. Patients with any contraindication to PDE5i, and/or PDE5i treatment in the past 1 month were excluded. Continuous hemodynamic monitoring was performed by placing a Swan-Ganz catheter. Information on cardiac index (CI), mean pulmonary arterial pressure (mPAP), mean systemic arterial pressure (mSAP), pulmonary arterial wedge pressure (PAWP), and pulmonary vascular resistance index (PVRI) was obtained for 4 hours after drug administration. RESULTS: The mPAP significantly decreased in both the U50 and U100 (−11 mmHg and −8 mmHg from baseline, respectively, p < 0.1). The mSAP also decreased in both U50 and U100; however, the decrease was greater in the U100 (Δ=−8.5 mmHg and Δ=−14.0 mmHg). CI increased in the U50, but decreased in the U100. Although PVRI decreased in both, statistical significance was only achieved in the U50 compared to placebo. PAWP was stable during monitoring. U50 had at least 4 hour-effect after administration. Only 2 patients with U100 experienced mild adverse events. CONCLUSIONS: This is the first demonstration of the acute hemodynamic changes induced by udenafil. U50 is considered an optimal dose for treating PAH with more than 4-hour treatment effect. TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT01553721.


Subject(s)
Arterial Pressure , Catheters , Connective Tissue Diseases , Cyclic Nucleotide Phosphodiesterases, Type 5 , Diagnosis , Erectile Dysfunction , Hemodynamics , Humans , Hypertension , Hypertension, Pulmonary , Male , Phosphodiesterase 5 Inhibitors , Pulmonary Wedge Pressure , Vascular Resistance
17.
Rev. cuba. reumatol ; 20(3): e83, sept.-dic. 2018.
Article in Spanish | LILACS, CUMED | ID: biblio-1093791

ABSTRACT

Introducción: Las enfermedades del tejido conectivo constituyen un problema de salud para las instituciones sanitarias de cualquier país, no solo por los síntomas que las acompañan, lo cual resulta motivo de atención médica frecuente, sino por la elevada carga asistencial, los elevados costos sanitarios y las afectaciones a la calidad de vida que genera en las personas que las padecen. Objetivo: Reflexionar sobre algunas enfermedades del tejido conectivo y sus complejidades morfológicas estructurales. Desarrollo: En la literatura consultada pudo constatarse la existencia de un grupo de enfermedades autoinmunes de tipo sistémico; tales como la artritis reumatoide, el lupus eritematoso sistémico, la esclerosis sistémica, la dermatomiositis, las miopatías inflamatorias, el síndrome antifosfolípido, el síndrome de Sjögren y las vasculitis necrotizantes sistémicas, que llaman la atención de los especialistas. Conclusiones: La mayoría de estas afecciones evolucionan con dolor, molestias y limitaciones para desarrollar las actividades de la vida cotidiana por lo que los pacientes suelen expresar sufrimiento, en particular, porque son enfermedades progresivas que pueden producir daños irreversibles con pronóstico no siempre favorable y cuya respuesta al tratamiento específico no siempre es satisfactoria, lo que acentúa el malestar y los síntomas en general y dan lugar a angustia y sufrimiento espiritual al paciente y sus familiares(AU)


Introduction: Connective tissue diseases are a health concern for healthcare institutions in any country, not only because of the symptoms they present, which is a reason for frequent medical attention, but also because of the need for care, high health costs and the great negative effects on the quality of life generated in the lives of people who suffer from them. Objective: To reflect about some connective tissue diseases and their structural and morphological complications. Development: During the analysis of the consulted literature, we could verify the existence of a group of systemic autoimmune diseases, such as rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, dermatomyositis, inflammatory myopathies, antiphospholipid syndrome, Sjögren's syndrome and systemic necrotizing vasculitis, which has drawn the specialists' attention. Conclusions: Most of these conditions develop with pain, discomfort and limitations to perform activities of daily life, a reason why patients often feel distressed, particularly because these are diseases that can cause irreversible damage with a not always favorable prognosis and whose response to specific treatment is not always satisfactory, which accentuates the discomfort and symptoms in general and gives rise to spiritual anguish and suffering in the patient and their relatives(AU)


Subject(s)
Humans , Arthritis, Rheumatoid , Scleroderma, Systemic , Connective Tissue Diseases , Dermatomyositis
18.
An. bras. dermatol ; 93(4): 573-575, July-Aug. 2018. graf
Article in English | LILACS | ID: biblio-949926

ABSTRACT

Abstract: Papular elastorrhexis is an acquired disease of elastic tissue; considered rare, its etiology and pathogenesis remain unknown. The vast majority of cases occur in women in the first or second decade of life. The disease manifests as multiple uniformly-sized, circumscribed, hypochromic and achromic papules located predominantly on the trunk and upper extremities. The lesions are generally asymptomatic and have a stable evolution over years. Its diagnosis is based on clinical and histopathological findings. The benignity of papular elastorrhexis and the subtlety of its clinical changes make the expectant treatment perfectly viable. The authors present one case in a young female patient.


Subject(s)
Humans , Female , Adult , Skin Diseases/pathology , Connective Tissue Diseases/pathology , Elastic Tissue/pathology
19.
Rev. méd. Chile ; 146(8): 938-942, ago. 2018. tab, graf
Article in Spanish | LILACS | ID: biblio-978779

ABSTRACT

Ehlers Danlos Syndrome comprises a heterogeneous group of genetic disorders of the connective tissue, due to defects in collagen or its modifying enzymes. We report a 21 years old male presenting with translucent skin revealing the subcutaneous venous pattern. He had a thin face, large-appearing eyes, thin lips, thin nose, joint hypermotility and history of hip dysplasia. A vascular Ehlers Danlos Syndrome was suspected. However, the genetic study to confirm the diagnosis was not done.


Subject(s)
Humans , Male , Adult , Young Adult , Ehlers-Danlos Syndrome/diagnosis , Genetic Heterogeneity , Connective Tissue Diseases/genetics , Connective Tissue Diseases/diagnostic imaging , Molecular Diagnostic Techniques , Ehlers-Danlos Syndrome/genetics
20.
Korean Leprosy Bulletin ; : 29-34, 2018.
Article in Korean | WPRIM | ID: wpr-718991

ABSTRACT

Calcinosis cutis is an uncommon disorder and defined as the deposit of insoluble calcium salts in the skin. It is classified as dystrophic, metastatic, idiopathic, and iatrogenic according to etiology. The dystrophic type is the most common and occurs in the previously damaged tissue, including connective tissue diseases, panniculitis, inherited disorders, benign and malignant tumors, a variety of scarring caused by burn, radiation, trauma, surgery, and keloid. Treatment options are limited. Whereas medical therapy is usually not very effective, surgical intervention has shown to be beneficial and is indicated when painful masses, recurrent infection, ulcerations, functional impairment, and cosmetic concerns exist. Herein, we report two cases of dystrophic calcinosis cutis associated with longstanding, painful, ulcerated cutaneous lesions in the extremities successfully treated with surgical excision.


Subject(s)
Burns , Calcinosis , Calcium , Cicatrix , Connective Tissue Diseases , Extremities , Keloid , Panniculitis , Salts , Skin , Ulcer
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