ABSTRACT
Sarcoidosis is one of the leading causes of inflammatory eye disease. All ocular structures can be affected, but uveitis is the main manifestation responsible for vision loss in ocular sarcoidosis. Typical sarcoid anterior uveitis presents with mutton-fat keratic precipitates, iris nodules, and posterior synechiae. Posterior involvement includes vitritis, vasculitis, and choroidal lesions. Cystoid macular edema is the most important and sight-threatening consequence of sarcoid uveitis. Patients with clinically isolated uveitis at diagnosis rarely develop other organ involvement. Even though, ocular sarcoidosis can have a severe impact on visual prognosis, early diagnosis and a wider range of available therapies (including intravitreal implants) have lessened the functional impact of the disease, particularly in the last decade. Corticosteroids are the cornerstone of treatment for sarcoidosis, but up to 30% of patients achieve remission with requiring high-dose systemic steroids. In these cases, the use of steroid-sparing immunosuppressive therapy (such as methotrexate) is unavoidable. Among these immunosuppressive treatments, anti TNF-α drugs have been a revolution in the management of non-infectious uveitis.
Subject(s)
Ophthalmologists , Sarcoidosis , Uveitis , Humans , Tumor Necrosis Factor Inhibitors/therapeutic use , Uveitis/diagnosis , Uveitis/etiology , Uveitis/drug therapy , Immunosuppressive Agents/therapeutic use , Vision Disorders/diagnosis , Sarcoidosis/complications , Sarcoidosis/diagnosisABSTRACT
Sarcoidosis can develop into a chronic disease in about 30% of cases. When general treatment is indicated, corticosteroids are the first-line treatment. More than one third of patients treated with corticosteroids receive a steroid-sparing agent. Although methotrexate is the most commonly used sparing agent, synthetic antimalarials have been used for more than fifty years on the basis of small, randomised, therapeutic trials. Despite this low level of evidence, chloroquine or more often hydroxychloroquine are used in daily practice, particularly to treat skin, bone and joint sarcoidosis, as well as hypercalcemia and certain types of uveitis. This review summarises the state of knowledge on steroid-sparing therapy in sarcoidosis, particularly in its extra-pulmonary form. These data support the need for good quality therapeutic trials to validate the use of hydroxychloroquine in this specific indication.
Subject(s)
Sarcoidosis, Pulmonary , Sarcoidosis , Adrenal Cortex Hormones/therapeutic use , Humans , Hydroxychloroquine/therapeutic use , Sarcoidosis/diagnosis , Sarcoidosis/drug therapy , Sarcoidosis, Pulmonary/drug therapy , Steroids/therapeutic useABSTRACT
In the past ten years, the better understanding of the pathophysiological mechanisms underlying inflammatory and autoimmune diseases has led to the emergence of many targeted therapies. Among them, the Janus kinase inhibitors are acting upstream in the inflammatory cascade of several key cytokines in disorders such as rheumatoid arthritis, ulcerative colitis or psoriasis. At the moment, these three diseases represent the only indications validated by the FDA and the EMA of the use of JAK inhibitors apart from hematology. Preclinical data and therapeutic trials indicate their efficacy in other autoimmune or inflammatory conditions, such as lupus, dermatomyositis, ankylosing spondylitis, sarcoidosis and giant cell arteritis. This review provides a summary of current use and advancement of knowledge in the use of JAK inhibitors in pathologies faced by internists.
Subject(s)
Internal Medicine/methods , Janus Kinase Inhibitors/therapeutic use , Arthritis, Rheumatoid/drug therapy , Autoimmune Diseases/drug therapy , Colitis, Ulcerative/drug therapy , Humans , Internal Medicine/trends , Protein Kinase Inhibitors/therapeutic use , Psoriasis/drug therapyABSTRACT
INTRODUCTION: First described in 1959, Hughes-Stovin syndrome is a very rare disorder combining vascular aneurysms, especially from pulmonary arteries, and thrombosis. The disease affects mostly the young male and is sometime associated with Behçet' disease. CASE REPORT: Here, we report the case of a 19-year-old man with hemoptysis and dyspnea revealing recurrent pulmonary embolisms despite efficient anticoagulant therapy. The patient subsequently developed fever and an inflammatory syndrome. Physical examination showed ulcers of the tongue. Angio-CT revealed recent pulmonary embolism, femoral vein thrombosis, and a unique threatening aneurysm of a left pulmonary artery segment. The aneurysm was embolized and simultaneously a vena cava filter was inserted. CONCLUSION: Hughes-Stovin syndrome requires immediate therapeutic decision, with an important risk of the anticoagulation. High dose steroids and in most cases, intensive immunosuppressive therapies are required such as cyclophosphamide.
Subject(s)
Aneurysm/diagnosis , Pulmonary Artery/pathology , Venous Thrombosis/diagnosis , Aneurysm/complications , Behcet Syndrome/complications , Behcet Syndrome/diagnosis , Behcet Syndrome/pathology , Dyspnea/diagnosis , Dyspnea/etiology , Hemoptysis/diagnosis , Hemoptysis/etiology , Humans , Male , Recurrence , Syndrome , Venous Thrombosis/complications , Young AdultABSTRACT
A detailed study has been made of hydrocarbons and fatty acids in nine surficial Mediterranean sediments. The hydrocarbon concentrations are generally low. The nature and the distribution of the hydrocarbons found in these sediments indicate that they originate from several different sources (terrestrial, biogenic, pyrolytic) and petroleum inputs are usually not the main source of hydrocarbons. Fatty acid concentrations vary in the range 2-52 mg.kg-1 dry sediment. Fatty acid distributions, between C14 and C20 show the biogenic autochthonous inputs. Branched and cyclopropanic fatty acids reflect a bacterial origin. These distributions are markedly different in oxic and anoxic sediments.
