The Experts below are selected from a list of 282 Experts worldwide ranked by ideXlab platform
Helmut Popper - One of the best experts on this subject based on the ideXlab platform.
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differential diagnosis and etiology of epitheloid cell Granulomatosis of the lung
Pathology Research and Practice, 2000Co-Authors: Helmut PopperAbstract:Our knowledge on epithelioid cell Granulomatosis of the lung has been extended in recent years. New entities have been added, like zirconiosis, others like tuberculosis, mycobacteriosis and sarcoidosis have gained new interest, because molecular techniques allowed new insight into their pathogenesis and a more rapid and species-specific diagnosis. Experimental work in addition has added a lot of information about the network of cytokines and other inflammatory mediators responsible for granuloma formation, however, our knowledge of this network is still incomplete. Three types of agents are now known to cause epitheloid cell granulomas: infectious organisms (bacteria, fungi, and parasites), products of plants and animals (pollen, sporangia, proteins), and metallic compounds. In addition there is still a group of epithelioid cell granulomatoses with unknown etiology. Sarcoidosis, one of these Granulomatosis, has recently elicited an old controversy: By molecular techniques Mycobacteria and Corynebacterium acnes have been identified in sarcoid granulomas and a link to the aetiology of sarcoidosis has been proposed. If these bacteria induce some cases of sarcoidosis by an allergic mechanism, has still to be proven.
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Epithelioid cell Granulomatosis of the lung: new insights and concepts.
Sarcoidosis Vasculitis and Diffuse Lung Diseases, 1999Co-Authors: Helmut PopperAbstract:: Our knowledge on epithelioid cell Granulomatosis of the lung has been extended in recent years. New entities have been added, like zirconiosis; others like tuberculosis, mycobacteriosis and sarcoidosis have gained new interest, because molecular techniques allowed new insight into their pathogenesis and a more rapid and species-specific diagnosis. Experimental work in addition has added a lot of information about the network of cytokines and other inflammatory mediators responsible for granuloma formation. However, our knowledge of this network is still incomplete. Three types of agents are now known to cause epithelioid cell granulomas: infectious organisms (bacteria and fungi), products of plants and animals (pollen, sporangia, proteins), and metallic compounds. In addition, there is still a group of epithelioid cell granulomatoses with unknown etiology. Sarcoidosis, one of these granulomatoses has recently elicited an old controversy; by molecular techniques atypical Mycobacteria and Corynebacterium acne have been identified in sarcoid granulomas and a link to the etiology of sarcoidosis has been proposed. Whether these bacteria induce some cases of sarcoidosis by an allergic mechanism, has still to be proven.
Peter A. Merkel - One of the best experts on this subject based on the ideXlab platform.
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Clinical Characteristics of an Internet-Based Cohort of Patient-Reported Diagnosis of Granulomatosis With Polyangiitis and Microscopic Polyangiitis: Observational Study.
Journal of medical Internet research, 2020Co-Authors: Jason Springer, Tanaz A Kermani, Antoine Sreih, Dianne G Shaw, Kalen Young, Cristina Burroughs, Peter A. MerkelAbstract:BACKGROUND Utilizing the traditional centers of excellence approach to conduct clinical trials involving rare diseases remains challenging. Patient-based registries have been shown to be both feasible and valid in several other diseases. OBJECTIVE This report outlines the clinical characteristics of a large internet registry cohort of participants with a self-reported diagnosis of Granulomatosis with polyangiitis or microscopic polyangiitis. METHODS Patients with a self-reported diagnosis of Granulomatosis with polyangiitis or microscopic polyangiitis in an internet-based prospective longitudinal cohort (from the Vasculitis Patient-Powered Research Network) were included. Data on symptoms, diagnostic testing, and treatment were collected using standardized questionnaires. RESULTS The study compared patients with Granulomatosis with polyangiitis (n=762) and patients with microscopic polyangiitis (n=164). Of the cohort, 97.7% (904/925) reported the diagnosis had been confirmed by a physician. Compared to microscopic polyangiitis, patients with Granulomatosis with polyangiitis reported significantly more ear, nose, and throat manifestations (Granulomatosis with polyangiitis: 641/723, 88.7%; microscopic polyangiitis: 89/164, 54.3%; z=10.42, P
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Clinical Characteristics of an Internet-Based Cohort of Patient-Reported Diagnosis of Granulomatosis With Polyangiitis and Microscopic Polyangiitis: Observational Study (Preprint)
2020Co-Authors: Jason Michael Springer, Tanaz A Kermani, Antoine Sreih, Dianne G Shaw, Kalen Young, Cristina M Burroughs, Peter A. MerkelAbstract:BACKGROUND Utilizing the traditional centers of excellence approach to conduct clinical trials involving rare diseases remains challenging. Patient-based registries have been shown to be both feasible and valid in several other diseases. OBJECTIVE This report outlines the clinical characteristics of a large internet registry cohort of participants with a self-reported diagnosis of Granulomatosis with polyangiitis or microscopic polyangiitis. METHODS Patients with a self-reported diagnosis of Granulomatosis with polyangiitis or microscopic polyangiitis in an internet-based prospective longitudinal cohort (from the Vasculitis Patient-Powered Research Network) were included. Data on symptoms, diagnostic testing, and treatment were collected using standardized questionnaires. RESULTS The study compared patients with Granulomatosis with polyangiitis (n=762) and patients with microscopic polyangiitis (n=164). Of the cohort, 97.7% (904/925) reported the diagnosis had been confirmed by a physician. Compared to microscopic polyangiitis, patients with Granulomatosis with polyangiitis reported significantly more ear, nose, and throat manifestations (Granulomatosis with polyangiitis: 641/723, 88.7%; microscopic polyangiitis: 89/164, 54.3%; <i>z</i>=10.42, <i>P</i><.001), fevers (Granulomatosis with polyangiitis: 325/588, 55.3%; microscopic polyangiitis: 64/139, 46.0%; <i>z</i>=1.96, <i>P</i>=.05), joint involvement (Granulomatosis with polyangiitis: 549/688, 79.8%; microscopic polyangiitis: 106/154, 68.8%; <i>z</i>=2.96, <i>P</i>=.003), and pulmonary involvement (Granulomatosis with polyangiitis: 523/734, 71.3%; microscopic polyangiitis: 90/154, 58.4%; <i>z</i>=3.13, <i>P</i>=.002). Compared to microscopic polyangiitis, patients with Granulomatosis with polyangiitis reported significantly less renal involvement (Granulomatosis with polyangiitis: 457/743, 61.5%; microscopic polyangiitis: 135/163, 82.8%; <i>z</i>=–5.18, <i>P</i><.001) and renal transplantation (Granulomatosis with polyangiitis: 10/721, 1.4%; microscopic polyangiitis: 7/164, 4.3%; <i>z</i>=–2.43, <i>P</i>=.02). Antineutrophil cytoplasmic antibody positivity was reported in 94.2% (652/692) of patients with Granulomatosis with polyangiitis and 96.1% (147/153) of patients with microscopic polyangiitis. A biopsy showing vasculitis was reported in 77.0% (562/730) of patients with Granulomatosis with polyangiitis and 81.9% (131/160) of patients with microscopic polyangiitis. CONCLUSIONS In this large, internet-based cohort of patients with a self-reported diagnosis of Granulomatosis with polyangiitis or microscopic polyangiitis, disease manifestations were consistent with expectations for each type of vasculitis. Given the rarity of these and other vasculitides, conducting some types of research through internet-based registries may provide an efficient alternative to inperson, center-of-excellence clinical trials.
Pamela M K Lutalo - One of the best experts on this subject based on the ideXlab platform.
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diagnosis and classification of Granulomatosis with polyangiitis aka wegener s Granulomatosis
Journal of Autoimmunity, 2014Co-Authors: Pamela M K Lutalo, David DcruzAbstract:Granulomatosis with polyangiitis (GPA, formerly known as Wegener's Granulomatosis) is an autoimmune small vessel vasculitis which is highly associated with anti-neutrophil cytoplasmic antibodies (ANCA). The hallmarks of this condition are systemic necrotising vasculitis, necrotising granulomatous inflammation, and necrotising glomerulonephritis. The aetiology of Granulomatosis with polyangiitis is linked to environmental and infectious triggers inciting onset of disease in genetically predisposed individuals. Anti-neutrophil cytoplasmic antibodies are pathogenic and play an important role in the pathogenesis of this disease, although ANCA positivity is not essential for a clinical diagnosis of Granulomatosis with polyangiitis. Granulomatosis with polyangiitis is diagnosed based on clinical manifestations of systemic vasculitis and histological evidence of necrotising vasculitis or granulomatous inflammation. This small vessel vasculitis may present as limited disease of the ears, nose and upper airways or mild, moderate or severe systemic disease. Immunosuppression and adjuvant therapies have contributed to the improved prognosis of Granulomatosis with polyangiitis over the past decades. Treatment strategies are tailored to the severity of the disease. They are based on published evidence of the efficacy and safety of the immunosuppressive drugs indicated to manage active vasculitis and maintain clinical remission. This review will summarise the history, aetiology, pathogenesis, classification, diagnosis and management of Granulomatosis with polyangiitis.
Winfried Göbel - One of the best experts on this subject based on the ideXlab platform.
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Vasculitis-like hemorrhagic retinal angiopathy in Wegener’s Granulomatosis
BMC research notes, 2013Co-Authors: Juliane Matlach, Florentina J. Freiberg, Ottar Gadeholt, Winfried GöbelAbstract:Background Granulomatosis with polyangiitis, also known as Wegener’s Granulomatosis, is a chronic systemic inflammatory disease that can also involve the eyes. We report a case of massive retinal and preretinal hemorrhages with perivascular changes as the initial signs in Granulomatosis with polyangiitis (Wegener’s Granulomatosis).
Paul E. Marik - One of the best experts on this subject based on the ideXlab platform.
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Lymphomatoid Granulomatosis: a rare cause of multiple pulmonary nodules.
Respiratory care, 2008Co-Authors: Bobbak Vahid, Daniel A. Salerno, Paul E. MarikAbstract:Lymphomatoid Granulomatosis is an uncommon disorder that can present as multiple pulmonary nodules. Systemic manifestations such as fever, weight loss, skin lesions, and other organ involvement may occur. The clinical presentation of lymphomatoid Granulomatosis can mimic infectious diseases (eg, tuberculosis), vasculitis, or metastatic malignancies.1 Lymphomatoid Granulomatosis should be considered in patients who present with pulmonary nodules and systemic symptoms.
