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John H Stone - One of the best experts on this subject based on the ideXlab platform.
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igg4 related Systemic Disease as a cause of idiopathic orbital inflammation including orbital myositis and trigeminal nerve involvement
Survey of Ophthalmology, 2012Co-Authors: Zachary S Wallace, Arezou Khosroshahi, Vikram Deshpande, Frederick A Jakobiec, Mark P Hatton, Jill Ritter, Judith A Ferry, John H StoneAbstract:IgG4-related Systemic Disease (IgG4-RD) is an inflammatory condition of unknown etiology that has been identified as the cause of tumefactive lesions in a number of tissues and organs. The role of the IgG4 remains to be clarified fully, but the histopathologic diagnosis hinges upon the finding of IgG4-bearing plasma cells in addition to characteristic morphologic features, with or without elevated seum IgG4. We present a 56-year-old man with orbital pseudotumor in whom, after 30 years of intractable Disease, biopsy showed IgG4-RD involving the lacrimal gland, extraocular muscles, intraconal fat, and trigeminal nerve. Six months after initiating treatment with rituximab, his Disease remained dormant, with improvement in his proptosis and normalization of serum IgG4 levels. We review the differential of idiopathic orbital inflammatory Disease, including IgG4-RD, and emphasize the need for biopsy for accurate diagnosis and to guide appropriate treatment.
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treatment approaches to igg4 related Systemic Disease
Current Opinion in Rheumatology, 2011Co-Authors: Arezou Khosroshahi, John H StoneAbstract:Purpose of reviewIgG4-related Systemic Disease (IgG4-RSD) is a Systemic fibroinflammatory condition that can affect any organ system. Prompt recognition and management of this Disease process are necessary to prevent sclerosis and permanent organ damage. Here, we review the advances in treatment app
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a clinical overview of igg4 related Systemic Disease
Current Opinion in Rheumatology, 2011Co-Authors: Arezou Khosroshahi, John H StoneAbstract:Purpose of reviewTo summarize the existing knowledge of various clinical presentations of IgG4-related Systemic Disease (IgG4-RSD) and to review the evolving list of organs affected by IgG4-RSD.Recent findingsThe term IgG4-RSD encompasses a variety of clinical entities once regarded as being entirel
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riedel s thyroiditis and multifocal fibrosclerosis are part of the igg4 related Systemic Disease spectrum
Arthritis Care and Research, 2010Co-Authors: Mollie Dahlgren, Arezou Khosroshahi, Petur G Nielsen, Vikram Deshpande, John H StoneAbstract:Objective Riedel's thyroiditis is a chronic fibrosing disorder of unknown etiology often associated with “multifocal fibrosclerosis.” IgG4-related Systemic Disease is characterized by IgG4+ plasma cell infiltration and fibrosis throughout many organs. We hypothesized that Riedel's thyroiditis is part of the IgG4-related Systemic Disease spectrum. Methods We searched our institution's pathology database using the terms “Riedel's,” “struma,” “thyroid,” and “fibrosis,” and identified 3 cases of Riedel's thyroiditis. Riedel's thyroiditis was diagnosed if there was a fibroinflammatory process involving all or a portion of the thyroid gland, with evidence of extension of the process into surrounding tissues. Immunohistochemical stains for IgG4 and IgG were performed. The histopathologic and immunohistochemical features of each involved organ were evaluated. The clinical features of one patient with multiple organ system Disease were described. Results All 3 thyroidectomy samples stained positively for IgG4-bearing plasma cells. One patient had extensive extrathyroidal involvement diagnostic of IgG4-related Systemic Disease, including cholangitis, pseudotumors of both the lung and lacrimal gland, and a lymph node contiguous to the thyroid that stained intensely for IgG4+ plasma cells. The histologic features of all organs involved were consistent with IgG4-related Systemic Disease. Patient 3 had 10 IgG4+ plasma cells per high-power field initially, but rebiopsy 2 years later demonstrated no IgG4+ plasma cells. That patient's second biopsy, characterized by fibrosis and minimal residual inflammation, further solidifies the link between IgG4-bearing plasma cells in tissue and the histologic evolution to Riedel's thyroiditis. Conclusion Riedel's thyroiditis is part of the IgG4-related Systemic Disease spectrum. In many cases, multifocal fibrosclerosis and IgG4-related Systemic Disease are probably the same entity.
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Rituximab therapy leads to rapid decline of serum IgG4 levels and prompt clinical improvement in IgG4-related Systemic Disease.
Arthritis & Rheumatism, 2010Co-Authors: Arezou Khosroshahi, Vikram Deshpande, D. Bloch, John H StoneAbstract:Objective. Patients with IgG4-related Systemic Disease (IgG4-RSD) frequently show an incomplete response to treatment with glucocorticoids and traditional Disease-modifying antirheumatic drugs (DMARDs). B lymphocyte depletion is a therapeutic strategy known to be effective for pemphigus vulgaris, an autoimmune condition mediated by IgG4 autoantibodies. This study was performed to assess the clinical and serologic responses to B lymphocyte depletion therapy with rituximab in patients with IgG4-RSD. Methods. Four patients with IgG4-RSD were treated with 2 intravenous doses (1 gram each) of rituximab. Clinical improvement was assessed by monitoring the tapering/discontinuation of prednisone and DMARDs, and by measuring the serum concentrations of B lymphocytes, immunoglobulins, and IgG subclasses before and after therapy. Results. Clinical features of IgG4-RSD in these 4 patients included autoimmune pancreatitis, sclerosing cholangitis, lymphoplasmacytic aortitis, salivary gland involvement, orbital pseudotumor, and lacrimal gland enlargement. The 3 patients with elevated serum IgG and IgG4 levels at baseline had a mean IgG concentration of 2,003 mg/dl (normal range 600–1,500 mg/dl) and a mean IgG4 concentration of 2,160 mg/dl (normal range 8–140 mg/dl). Among these patients, the serum IgG4 concentrations declined by a mean of 65% within 2 months of rituximab administration. All 4 patients demonstrated striking clinical improvement within 1 month of the initiation of rituximab therapy, and tapering or discontinuation of their treatment with prednisone and DMARDs was achieved in all 4 patients. A decrease in IgG concentration was observed for the IgG4 subclass only. Conclusion. Treatment with rituximab led to prompt clinical and serologic improvement in these patients with refractory IgG4-RSD, and is a viable treatment option for this condition. The decline in serum IgG4 concentrations was substantially steeper than that of the autoantibody concentrations in immune-mediated conditions in which rituximab is effective, such as in rheumatoid arthritis. In addition, the reduction in IgG-subclass levels appeared to be specific for IgG4. The swift improvement of IgG4-RSD suggests that rituximab achieves its effects in IgG4-RSD by depleting the pool of B lymphocytes that replenish short-lived IgG4-secreting plasma cells.
Arezou Khosroshahi - One of the best experts on this subject based on the ideXlab platform.
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igg4 related Systemic Disease as a cause of idiopathic orbital inflammation including orbital myositis and trigeminal nerve involvement
Survey of Ophthalmology, 2012Co-Authors: Zachary S Wallace, Arezou Khosroshahi, Vikram Deshpande, Frederick A Jakobiec, Mark P Hatton, Jill Ritter, Judith A Ferry, John H StoneAbstract:IgG4-related Systemic Disease (IgG4-RD) is an inflammatory condition of unknown etiology that has been identified as the cause of tumefactive lesions in a number of tissues and organs. The role of the IgG4 remains to be clarified fully, but the histopathologic diagnosis hinges upon the finding of IgG4-bearing plasma cells in addition to characteristic morphologic features, with or without elevated seum IgG4. We present a 56-year-old man with orbital pseudotumor in whom, after 30 years of intractable Disease, biopsy showed IgG4-RD involving the lacrimal gland, extraocular muscles, intraconal fat, and trigeminal nerve. Six months after initiating treatment with rituximab, his Disease remained dormant, with improvement in his proptosis and normalization of serum IgG4 levels. We review the differential of idiopathic orbital inflammatory Disease, including IgG4-RD, and emphasize the need for biopsy for accurate diagnosis and to guide appropriate treatment.
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treatment approaches to igg4 related Systemic Disease
Current Opinion in Rheumatology, 2011Co-Authors: Arezou Khosroshahi, John H StoneAbstract:Purpose of reviewIgG4-related Systemic Disease (IgG4-RSD) is a Systemic fibroinflammatory condition that can affect any organ system. Prompt recognition and management of this Disease process are necessary to prevent sclerosis and permanent organ damage. Here, we review the advances in treatment app
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a clinical overview of igg4 related Systemic Disease
Current Opinion in Rheumatology, 2011Co-Authors: Arezou Khosroshahi, John H StoneAbstract:Purpose of reviewTo summarize the existing knowledge of various clinical presentations of IgG4-related Systemic Disease (IgG4-RSD) and to review the evolving list of organs affected by IgG4-RSD.Recent findingsThe term IgG4-RSD encompasses a variety of clinical entities once regarded as being entirel
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riedel s thyroiditis and multifocal fibrosclerosis are part of the igg4 related Systemic Disease spectrum
Arthritis Care and Research, 2010Co-Authors: Mollie Dahlgren, Arezou Khosroshahi, Petur G Nielsen, Vikram Deshpande, John H StoneAbstract:Objective Riedel's thyroiditis is a chronic fibrosing disorder of unknown etiology often associated with “multifocal fibrosclerosis.” IgG4-related Systemic Disease is characterized by IgG4+ plasma cell infiltration and fibrosis throughout many organs. We hypothesized that Riedel's thyroiditis is part of the IgG4-related Systemic Disease spectrum. Methods We searched our institution's pathology database using the terms “Riedel's,” “struma,” “thyroid,” and “fibrosis,” and identified 3 cases of Riedel's thyroiditis. Riedel's thyroiditis was diagnosed if there was a fibroinflammatory process involving all or a portion of the thyroid gland, with evidence of extension of the process into surrounding tissues. Immunohistochemical stains for IgG4 and IgG were performed. The histopathologic and immunohistochemical features of each involved organ were evaluated. The clinical features of one patient with multiple organ system Disease were described. Results All 3 thyroidectomy samples stained positively for IgG4-bearing plasma cells. One patient had extensive extrathyroidal involvement diagnostic of IgG4-related Systemic Disease, including cholangitis, pseudotumors of both the lung and lacrimal gland, and a lymph node contiguous to the thyroid that stained intensely for IgG4+ plasma cells. The histologic features of all organs involved were consistent with IgG4-related Systemic Disease. Patient 3 had 10 IgG4+ plasma cells per high-power field initially, but rebiopsy 2 years later demonstrated no IgG4+ plasma cells. That patient's second biopsy, characterized by fibrosis and minimal residual inflammation, further solidifies the link between IgG4-bearing plasma cells in tissue and the histologic evolution to Riedel's thyroiditis. Conclusion Riedel's thyroiditis is part of the IgG4-related Systemic Disease spectrum. In many cases, multifocal fibrosclerosis and IgG4-related Systemic Disease are probably the same entity.
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Rituximab therapy leads to rapid decline of serum IgG4 levels and prompt clinical improvement in IgG4-related Systemic Disease.
Arthritis & Rheumatism, 2010Co-Authors: Arezou Khosroshahi, Vikram Deshpande, D. Bloch, John H StoneAbstract:Objective. Patients with IgG4-related Systemic Disease (IgG4-RSD) frequently show an incomplete response to treatment with glucocorticoids and traditional Disease-modifying antirheumatic drugs (DMARDs). B lymphocyte depletion is a therapeutic strategy known to be effective for pemphigus vulgaris, an autoimmune condition mediated by IgG4 autoantibodies. This study was performed to assess the clinical and serologic responses to B lymphocyte depletion therapy with rituximab in patients with IgG4-RSD. Methods. Four patients with IgG4-RSD were treated with 2 intravenous doses (1 gram each) of rituximab. Clinical improvement was assessed by monitoring the tapering/discontinuation of prednisone and DMARDs, and by measuring the serum concentrations of B lymphocytes, immunoglobulins, and IgG subclasses before and after therapy. Results. Clinical features of IgG4-RSD in these 4 patients included autoimmune pancreatitis, sclerosing cholangitis, lymphoplasmacytic aortitis, salivary gland involvement, orbital pseudotumor, and lacrimal gland enlargement. The 3 patients with elevated serum IgG and IgG4 levels at baseline had a mean IgG concentration of 2,003 mg/dl (normal range 600–1,500 mg/dl) and a mean IgG4 concentration of 2,160 mg/dl (normal range 8–140 mg/dl). Among these patients, the serum IgG4 concentrations declined by a mean of 65% within 2 months of rituximab administration. All 4 patients demonstrated striking clinical improvement within 1 month of the initiation of rituximab therapy, and tapering or discontinuation of their treatment with prednisone and DMARDs was achieved in all 4 patients. A decrease in IgG concentration was observed for the IgG4 subclass only. Conclusion. Treatment with rituximab led to prompt clinical and serologic improvement in these patients with refractory IgG4-RSD, and is a viable treatment option for this condition. The decline in serum IgG4 concentrations was substantially steeper than that of the autoantibody concentrations in immune-mediated conditions in which rituximab is effective, such as in rheumatoid arthritis. In addition, the reduction in IgG-subclass levels appeared to be specific for IgG4. The swift improvement of IgG4-RSD suggests that rituximab achieves its effects in IgG4-RSD by depleting the pool of B lymphocytes that replenish short-lived IgG4-secreting plasma cells.
Sujata R Harshad - One of the best experts on this subject based on the ideXlab platform.
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subcutaneous sarcoidosis is it a specific subset of cutaneous sarcoidosis frequently associated with Systemic Disease
Journal of The American Academy of Dermatology, 2006Co-Authors: Iftikhar Ahmed, Sujata R HarshadAbstract:Background Skin is involved in 25% of cases of sarcoidosis. The lesions are specific and nonspecific depending on the presence or absence of granulomas, respectively. Specific lesions are not thought to have prognostic significance and are not associated with Systemic Disease. Objective We sought to evaluate for the presence or absence of Systemic Disease in patients with subcutaneous sarcoidosis. Methods With diagnostic criteria of subcutaneous sarcoidosis, 33 cases were identified in the literature and 21 cases in our institutional database. A retrospective clinical and pathologic review of these cases was conducted. Results Subcutaneous sarcoidosis is characterized by a peak incidence during the fourth decade; female predisposition; asymptomatic to slightly tender lesions typically involving the upper extremities; cutaneous lesional clustering and multiplicity; autoimmune Disease associations at time of diagnosis in a subset of cases; Systemic Disease associations at diagnosis in most patients, typically consisting of bilateral hilar adenopathy; and a favorable response to oral corticosteroid therapy. Limitations Retrospective analysis with inadequate documentation of therapeutic regimens and their responses in some cases is a limitation of this study. Conclusions The confirmatory diagnosis of subcutaneous sarcoidosis depends on identifying pannicular noninfectious sarcoidal or epithelioid granulomas with minimal lymphocytic inflammation. Subcutaneous sarcoidosis is the only specific subset of cutaneous sarcoidosis frequently associated with Systemic Disease.
Sergio Casasflores - One of the best experts on this subject based on the ideXlab platform.
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the epl1 and sm1 proteins from trichoderma atroviride and trichoderma virens differentially modulate Systemic Disease resistance against different life style pathogens in solanum lycopersicum
Frontiers in Plant Science, 2015Co-Authors: Miguel Angel Salasmarina, Maria I Isordiajasso, Maria Auxiliadora Islasosuna, Pablo Delgadosanchez, Juan Francisco Jimenezbremont, Margarita Rodriguezkessler, Maria Teresa Rosalessaavedra, Alfredo Herreraestrella, Sergio CasasfloresAbstract:Fungi belonging to the genus Trichoderma, commonly found in soil or colonizing plant roots, exert beneficial effects on plants, including the promotion of growth and the induction of resistance to Disease. T. virens and T. atroviride secrete the proteins Sm1 and Epl1, respectively, which elicit local and Systemic Disease resistance in plants. In this work, we show that these fungi promote growth in tomato (Solanum lycopersicum) plants. T. virens was more effective than T. atroviride in promoting biomass gain, and both fungi were capable of inducing Systemic protection in tomato against Alternaria solani, Botrytis cinerea, and Pseudomonas syringae pv. tomato (Pst DC3000). Deletion (KO) of epl1 in T. atroviride resulted in diminished Systemic protection against A. solani and B. cinerea, whereas the T. virens sm1 KO strain was less effective in protecting tomato against Pst DC3000 and B. cinerea. Importantly, overexpression (OE) of epl1 and sm1 led to an increase in Disease resistance against all tested pathogens. Although the Trichoderma WT strains induced both Systemic acquired resistance (SAR)- and induced Systemic resistance (ISR)-related genes in tomato, inoculation of plants with OE and KO strains revealed that Epl1 and Sm1 play a minor role in the induction of these genes. However, we found that Epl1 and Sm1 induce the expression of a peroxidase and an α-dioxygenase encoding genes, respectively, which could be important for tomato protection by Trichoderma spp. Altogether, these observations indicate that colonization by beneficial and/or infection by pathogenic microorganisms dictates many of the outcomes in plants, which are more complex than previously thought.
Eckhard Thiel - One of the best experts on this subject based on the ideXlab platform.
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detection of subclinical Systemic Disease in primary cns lymphoma by polymerase chain reaction of the rearranged immunoglobulin heavy chain genes
Journal of Clinical Oncology, 2006Co-Authors: Kristoph Jahnke, Michael Hummel, Agnieszka Korfel, Thomas Burmeister, Philipp Kiewe, H A Klasen, Hanshenning Muller, Harald Stein, Eckhard ThielAbstract:Purpose To search for subclinical Systemic Disease in bone marrow and peripheral blood in patients with primary CNS lymphoma (PCNSL) to elucidate whether extracerebral relapse may represent a sequel of initial occult Systemic Disease rather than true extracerebral spread. Patients and Methods Bone marrow and peripheral-blood specimens of 24 PCNSL patients were examined using polymerase chain reaction (PCR) for analysis of clonally rearranged immunoglobulin heavy-chain (IgH) genes. Results Identical dominant PCR products were found in bone marrow aspirates, blood samples, and tumor biopsy specimens of two patients, indicating that the same tumor cell population is present in the CNS and in extracerebral sites. Follow-up IgH PCR performed in one of these patients in complete remission 24 months after diagnosis yielded a persistent monoclonal product in the blood. An oligoclonal IgH rearrangement pattern was found in the tumor specimen of two other patients, whereas bone marrow and blood samples demonstrated...
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detection of subclinical Systemic Disease in primary central nervous system lymphoma pcnsl by polymerase chain reaction pcr of the rearranged immunoglobulin heavy chain igh genes
Journal of Clinical Oncology, 2006Co-Authors: Kristoph Jahnke, Michael Hummel, Agnieszka Korfel, Thomas Burmeister, Harald Stein, H Mueller, Claudia Seide, Eckhard ThielAbstract:1533 Background: An unresolved question is why some PCNSL spread Systemically while most others do not. It was postulated that extracerebral relapse of PCNSL may represent a sequel of initial occult Systemic Disease rather than true extracerebral spread. Methods: To elucidate this question, we examined bone marrow and peripheral blood specimens of 24 patients with newly diagnosed PCNSL using PCR for the presence of clonally rearranged IgH genes. The applied IgH PCR method was recently developed by a European Concerted Action (BioMed-2). To all samples (50 ng DNA), three different FR primer sets (FR1, FR2 and FR3) were applied in conjunction with a JH consensus primer (JH22). Baseline routine staging procedures showed no evidence of Systemic lymphoma manifestations in all patients. Results: The same dominant PCR products were found in bone marrow aspirates, blood samples and tumor biopsy specimens from three patients, indicating the presence of the same tumor cell population in the CNS as well as in extrac...
