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العنوان
Pulmonary Manifestions of Rheumatic Diseases\
الناشر
Ain Shams university.
المؤلف
Ebeid,Aly Mahmoud.
هيئة الاعداد
مشرف / Sherin Mohamed Hosny.
مشرف / Howaida Elsayed Mansour
مشرف / Abd El-Azeim Mohammad Alhefny
باحث / Aly Mahmoud Ebeid
الموضوع
Pulmonary Manifestions. Rheumatic Diseases.
تاريخ النشر
2011
عدد الصفحات
p.:197
اللغة
الإنجليزية
الدرجة
ماجستير
التخصص
الطب الباطني
تاريخ الإجازة
1/1/2011
مكان الإجازة
جامعة عين شمس - كلية الطب - Internal Medicine
الفهرس
Only 14 pages are availabe for public view

from 197

from 197

Abstract

Rheumatological disorders are a heterogeneous group of autoimmune disorders of unknown etiology. They involve the lungs directly or as a complication of treatment.
The incidence of pulmonary involvement is increasing in Rheumatological disorders patients. This can be mainly attributed to the increased use of invasive techniques and, in part, to the use of high-resolution computed tomography (HRCT) and pulmonary function tests (PFTs). Because the prognosis, degree of reversibility, and optimal therapy differ for each disease presentation, a thorough knowledge of the pulmonary clinical picture of each rheumatic disease is important.
The pleuropulmonary manifestations of SLE range in severity from the minor pleuritic pain of serositis to the life threatening consequences of pulmonary haemorrhage.
Early detection of pulmonary disease in a patient with SLE is of importance because therapy ranges from aggressive immunosuppression for lupus pneumonitis, to anticoagulation for antiphospholipid antibody syndrome, to lowering immunosuppression while starting antimicrobial therapy in settings where infection is the culprit.
Treatment strategies for pulmonary disease in SLE are based on limited data and the experience from other connective tissue disorders. Continued investigations into the pathogenesis and treatment of SLE-related lung involvement are needed to improve morbidity and mortality outcomes for patients with SLE.
A variety of pulmonary manifestations are associated with RA that influences morbidity and increases the risk of mortality. Recent advances in HRCT reveal basilar ground-glass and reticulonodular opacities, which along with supporting clinical and physiologic data in asymptomatic patients with RA now suggest the earlier diagnosis.
The majority of patients with progressive pulmonary symptoms, when treated with corticosteroids have equivocal results. Only few case reports document the efficacy of immunosuppressive or cytotoxic medications.
There is paucity of evidence of the efficacy of treatment approaches to RA-related ILD. Randomized, controlled clinical trials are required to better understand the pathophysiology and response to therapy to improve the outcomes of patients with these conditions.
Because ILD is a frequent manifestation of Idiopathic inflammatory myopathies (polymyositis and dermatomyositis), and this manifestation of the disease is associated with increased morbidity and mortality, investigations to detect pulmonary involvement should be performed during the initial evaluation as well as during follow-up.
This evaluation should include chest radiography, HRCT of the lungs, and pulmonary function tests. The relatively low incidence and prevalence of many forms of ILD has hampered attempts at performing adequately powered and soundly designed clinical trials to evaluate pharmacologic treatments for these disorders. More insight into the etiopathogenesis from ongoing clinical trials is likely, and additional well-designed prospective studies are necessary to answer the questions of optimal treatment strategies for all forms of autoimmune ILD.
Sjögren’s syndrome (SS) is a complex autoimmune exocrinopathy with multifactorial pathogenesis and multisystem manifestation. It is called primary Sjögren’s syndrome (PSS) when the manifestations are seen without any other co-existent rheumatic diseases. Respiratory system manifestations are protean; upper airway symptoms are very common and so is the complaint of dry cough.
The PSS patients may develop interstitial lung diseases (ILDs) such as usual interstitial pneumonia (UIP), non-specific interstitial pneumonia (NSIP), lymphocytic interstitial pneumonia (LIP), bronchiolitis obliterans and organising pneumonia (BOOP), etc. They may also develop the whole spectrum of lymphoproliferative disorders of the lung ranging from LIP to follicular bronchiolitis, nodular lymphoid hyperplasia and low-grade lymphomas.
Therapeutic options include symptomatic and supportive measures and corticosteroids as the mainstay of the treatment for ILDs occurring in these patients. Pulmonary involvement may be a source of significant morbidity in these patients, though only rarely, it is the cause of death.
The respiratory system may be involved in all systemic vasculitides, although with a variable frequency. Lung disease is a very common and important feature of the antineutrophil cytoplasmic antibodies (ANCA)-associated systemic vasculitides.
The involvement of the respiratory system is a very common and important feature of the antineutrophil cytoplasmic antibodies (ANCA)-associated systemic vasculitides, whereas is less frequent in other Systemic vasculitides. It comprises a wide spectrum of clinical features and radiological findings, and may have a prognostic significance. The assessment of the respiratory system should be included in the work-up of all patients with systemic vasculitides, especially of those with antineutrophil cytoplasmic antibodies (ANCA)-associated systemic vasculitides.
SSc and MCTD represent a heterogeneous, complex group of disorders that have important pulmonary manifestations. Patients may present initially with only dyspnea as their chief complaint. The clinician must recognize the importance of screening for Rheumatological disorders in any patient presenting with ILD or PAH.
Treatment benefits consist of the prevention of progression and are largely confined to patients with extensive pulmonary fibrosis. Accurate prognostic evaluation by staging the severity of lung disease remains central to management and will be a major focus of future studies.
Sarcoidosis is a relatively common disease with characteristic imaging findings. However, a diagnosis might be difficult for several reasons. These reasons include nonspecific clinical features and difficulty in the histopathological differentiation from granulomatous infections such as tuberculosis. In addition, atypical manifestations on radiological images can make diagnosis difficult.
Radiographic features and lung function impairments are supportive in managing how serious the disease is in an individual patient but are not the parameters for definite prognosis.
We recommend to investigate respiratory system and follow up with Pulmonary function tests in all patients with rheumatic diseases, for early detection and so better management of pulmonary manifestations or complications.