Seminars in respiratory and critical care medicine
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Fluorodeoxyglucose (FDG), labeled with a positron emitting fluorine-18 ((18)F), is a synthesized glucose analogue and is well known for its application in a wide variety of clinical conditions such as cancer. Visualizing metabolic activity of inflammation is another application of FDG in positron emission tomography (PET). Here, active granulomas appear to have a high affinity for FDG, which is reflected in a high sensitivity of FDG PET imaging. ⋯ Moreover, FDG PET activity in lung parenchyma correlates with decrease of lung function values over time. Also in cardiac involvement in sarcoidosis, FDG PET is a promising technique complementary to magnetic resonance imaging, especially in guiding treatment. New developments, such as applications for quantitative organ-specific measurement, are proceeding and will probably enhance the clinical implementation of FDG PET in sarcoidosis.
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Semin Respir Crit Care Med · Jun 2014
ReviewCardiac involvement in sarcoidosis: evolving concepts in diagnosis and treatment.
Clinically evident sarcoidosis involving the heart has been noted in at least 2 to 7% of patients with sarcoidosis, but occult involvement is much higher (> 20%). Cardiac sarcoidosis is often not recognized antemortem, as sudden death may be the presenting feature. Cardiac involvement may occur at any point during the course of sarcoidosis and may occur in the absence of pulmonary or systemic involvement. ⋯ Although randomized therapeutic trials have not been done, corticosteroids (alone or combined with additional immunosuppressive medications) remain the mainstay of treatment. Because of the potential for sudden cardiac death, implantable cardioverter-defibrillators should be placed in any patient with cardiac sarcoidosis and serious ventricular arrhythmias or heart block, and should be considered for cardiomyopathy. Cardiac transplantation is a viable option for patients with end-stage cardiac sarcoidosis refractory to medical therapy.
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For treatment of sarcoidosis, one should develop a long-term management plan. Factors to be considered include the organ involvement and severity of symptoms. Different organ manifestations may require different treatments. ⋯ These include cytotoxic agents such as methotrexate, biologic agents such as the antitumor necrosis factor antibody infliximab, and other anti-inflammatory drugs such as hydroxychloroquine. In some cases, anti-inflammatory drugs may not help. Examples include pulmonary hypertension and fibrosis.
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Semin Respir Crit Care Med · Jun 2014
ReviewSarcoidosis and common variable immunodeficiency: similarities and differences.
Common variable immunodeficiency (CVID) is a primary immunodeficiency that is characterized by hypogammaglobulinemia and poor/absent specific antibody production. Granulomatous and lymphocytic interstitial lung disease (GLILD) is an increasingly recognized complication of CVID, occurring in 10 to 20% of patients. GLILD is characterized by non-necrotizing granuloma, lymphocytic interstitial pneumonitis and follicular bronchiolitis-histological patterns that are typically present in the same biopsy. ⋯ The presence of noncaseating granuloma in the lung along with some of the extrapulmonary features of GLILD may lead to an incorrect diagnosis of sarcoidosis. However, GLILD differs from sarcoidosis in several important ways including mode of presentation, extrapulmonary manifestations, radiographic abnormalities on high-resolution computed tomography scan of the chest, and laboratory features (serum immunoglobulins, bronchoalveolar lavage, and histopathology). The misdiagnosis of sarcoidosis in a patient with CVID and GLILD can lead to inappropriate treatment and increase the morbidity and mortality of the disorder.