Pulmonary arterial hypertension associated with congenital heart disease: lessons learnt from the large Turkish Nationwide Registry (THALES) (original) (raw)
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Cardiac manifestations of IgG4 related disease; a case series
European Heart Journal - Case Reports
Background IgG4-related disease (IgG4-RD) is an autoimmune condition affecting almost every organ system, with an early inflammatory phase and later fibrotic consequences. Vascular manifestations, particularly, large-vessel involvement in IgG4-RD, are well described. However, important IgG4-related effects on medium-sized arteries and the pericardium are less well recognized. These less frequently reported cardiovascular effects of IgG4-RD include coronary artery stenosis, pericardial disease, cardiac masses, and valvular heart disease. Case Summary This case series focuses on three patients that demonstrate the cardiovascular effects of IgG4-RD and the pitfalls and importance of early diagnosis. Cases 1 and 2 presented with cardiac manifestations prior to more typical organ systems being affected which led to a delay in diagnosis. Case 1 presented with an acute myocardial infarction secondary to IgG4-RD of the coronary arteries and Case 2 presented with pericarditis which progresse...
IgG4-related disease (IgG4-RD) is a recently recognized systemic disease characterized by tumefactive lesions in various organ systems. The list of organs that can be involved continues to expand, and recently computed tomography (CT) descriptions of the pulmonary lesions found in the disease have been described. The clinical symptoms are nonspecific and may include cough, dyspnea, chest pain, and fever. The appropriate clinical presentation along with elevated serum IgG4 concentrations and pathologic evidence of lymphoplasmacytic infiltrates with abundant IgG4-positive plasma cells and storiform fibrosis is consistent with the disease. Steroids are used to treat this disease in addition to immunosupressives such as cyclosporine or rituxumab for steroid refractory disease. The pulmonary manifestations and imaging features can often mimic malignancy, and as such knowledge of the diagnostic, clinicopathologic, and radiographic features of the disease is required in order to provide appropriate diagnostic workup and treatment.
IgG4-Related Cardiovascular Disorders
International Heart Journal, 2014
Immunoglobulin4 (IgG4)-related disease is a systemic inflammatory disease characterized by elevation of serum IgG4. It involves various organs such as the pancreas (autoimmune pancreatitis), lacrimal gland (Mikulicz's disease), retroperitoneum (retroperitoneal fibrosis), aorta (aortic aneurysm and aortitis), heart (constrictive pericarditis), and pseudotumors around the coronary arteries. These disorders often coexist in accordance with progression of the disease. Because IgG4-related cardiovascular disorder affects the patient's prognosis, early detection and treatment is important. Coronary CT imaging and echocardiography accidentally detect IgG4-related disorders and 18 FDG-PET imaging can identify active inflammation in the lesions. Measurement of serum IgG4 levels and tissue biopsy are necessary for diagnosis. Minor salivary gland biopsy is recommended even though 18 FDG uptake is not detected when it is difficult to obtain a biopsy specimen from IgG4-related cardiovascular lesions. The first-line treatment is high-dose corticosteroid therapy, however, relapse is often reported. Corticosteroids suppress the development of active inflammatory diseases such as aortitis, pericarditis, and pseudotumors, but already-developed lesions do not respond. A large developed aneurysm can rupture even during or after corticosteroid therapy, therefore, additional surgical treatment may be needed. Treatment of IgG4-related cardiovascular disorders might require higher doses of corticosteroids than IgG4-related extracardiovascular disorders. The adequate dose of corticosteroid, type and dose of immunosuppressant, and surgical intervention should be carefully considered on a case-by-case basis.
Pleomorphic Pulmonary Manifestations of IgG4-Related Disease
Case Reports in Rheumatology, 2019
Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory disorder which has been first reported in 2001 by Hamano and colleagues in a patient with autoimmune sclerosing pancreatitis. Almost every organ in the human body can be affected by IgG4-RD from infiltration with IgG4-positive plasma cells. Involvement of lungs with IgG4 is reported previously, but still, there is no clear picture of the pathophysiology behind lung involvement. Here, we are presenting a patient who has IgG4-RD presenting as pseudotumor of the lungs.
IgG4-related disease can present as recurrent spontaneous hemothorax: a case report
BMC Pulmonary Medicine, 2019
Background: Immunoglobulin G4-related disease (IgG4-RD) encompasses a group of immune-mediated disorders that are gaining increasing recognition. Pulmonary presentations are common, with four types of patterns been described on radiography, including solid nodular, bronchovascular, ground glass opacities, and alveolar interstitial. Pleural thickening and pleural effusion have also been reported. However, there have been no reports of IgG4-RD that presents as spontaneous hemothorax. Case presentation: A 61-year-old Chinese woman experienced recurrent right-sided chest pain and transient syncope. A significant decrease in her hemoglobin level and thick bloody pleural fluid demonstrated spontaneous hemothorax. The elevated serum IgG4 and histopathological analysis of the right pleura and intercostal lymph node specimens all supported the diagnosis of IgG4-RD in this patient. Further diagnostic evaluation did not reveal other causes for spontaneous hemothorax. She received steroids and no recurrent bleeding event occurred during a follow-up period of more than 1 year. Conclusion: Recurrent spontaneous hemothorax can be a rare manifestation of IgG4-RD, with pleural involvement as the most probable mechanism.
Primary Pulmonary Arterial Hypertension and Autoimmune Polyendocrine Syndrome in a Pediatric Patient
Pediatric Cardiology, 2010
Both primary pulmonary artery hypertension (PPAH) and autoimmune polyendocrine syndrome (APS) are rare disorders in children. We report a boy who was diagnosed with severe PPAH at 12 years of age. He was treated with prostacyclin for 6 years, briefly with adjunct bosentan, and eventually sildenafil was added. Six years later, after his diagnosis of PPAH, he developed APS in the form of hyperthyroidism and type 1 diabetes mellitus. No mutations were identified through genetic testing of bone morphogenetic protein receptor type II and the autoimmune-regulator gene. To our knowledge this is the first description of the combination of these two extremely rare diseases in a child.
International journal of cardiology, 2014
IgG4-related disease (IgG4-RD) is a novel fibroinflammatory condition. Identified in 2003, it has been described in many organ systems. It is associated with retroperitoneal fibrosis, autoimmune pancreatitis, and hepatopathy. Cardiac manifestations are rare. Type-2 helper T cell immune-mediated responses are thought to be pathogenic. Serum IgG4 levels are often elevated, however immunohistochemical analysis is required for diagnosis. We report a case of IgG4-RD with effusive-constrictive pericarditis, cardiac tamponade and hepatopathy in the setting of normal serum IgG4 levels. This has not been previously reported in the literature. A 75 year old male with a past medical history significant for reactive airway disease and seasonal allergies presented for evaluation of scrotal swelling. He denied provocative or palliative factors. He reported associated dyspnea that worsens with exertion. Additionally, he reported lower extremity edema, orthopnea, paroxysmal nocturnal dyspnea, and a dry cough. He denied chest pain, palpitations, claudication, or syncope. He had no fever, chills, nausea, emesis, or dysuria. He denied recent travel, or occupational exposures. He immigrated to the United States from the Ukraine. Physical examination was notable for tachycardia. Jugular venous pressure was 12 cm of water. Cardiac auscultation revealed a normal S1 and S2, without murmurs, rubs, gallops, or clicks. No pericardial knock or friction rub was noted. There was no evidence of pulsus paradoxus. Point of maximal impulse was non-displaced. Lungs were clear to auscultation bilaterally. Abdomen was soft and non-tender. Lower extremity pitting edema was present bilaterally. An electrocardiogram revealed atrial flutter with 2:1 atrioventricular block and a new right bundle branch block. Transthoracic echocardiography was performed and revealed mild to moderate concentric left ventricular hypertrophy with atypical ☆ This manuscript is an original work, and it is not under consideration for publication elsewhere. All authors meet criteria for authorship. There are no affiliations, financial disclosures, or conflicts of interest to report. No funding was obtained.
IgG4-related respiratory disease
Modern Rheumatology, 2019
IgG4-related diseases (IgG4-RDs), such as autoimmune pancreatitis and IgG4-related Mikulicz disease, are often accompanied by intrathoracic lesions, which are called IgG4-related respiratory disease (IgG4-RRD). IgG4-RRD has few subjective symptoms, and is usually detected during workup of patients with extra-thoracic lesions of IgG4-RD. IgG4-RRD is characterized by various conditions, including masses, nodules, thickening, and infiltration at numerous sites in the thorax through lymphatic routes. Although elevated serum IgG4 concentrations and pathologic evidence of lymphoplasmacytic infiltrates with abundant IgG4-positive plasma cells are characteristic findings of IgG4-RD, other intrathoracic diseases, such as multicentric Castleman disease and malignancy, may present with similar findings. Developing diagnostic criteria for IgG4-RRD, including clinicoradiological and pathological characteristics, is necessary for its appropriate diagnosis.