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John H. Stone - One of the best experts on this subject based on the ideXlab platform.
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A clinical overview of IgG4-related Systemic Disease.
Current Opinion in Rheumatology, 2020Co-Authors: Arezou Khosroshahi, John H. StoneAbstract:PURPOSE OF REVIEW: To 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 FINDINGS: The term IgG4-RSD encompasses a variety of clinical entities once regarded as being entirely separate Diseases. The list of organs associated with this condition is growing steadily. Tissue biopsies reveal striking histopathological similarity, regardless of which organ is involved, although subtle differences across organs exist. Diffuse lymphoplasmacytic infiltrates, presence of abundant IgG4-positive plasma cells and extensive fibrosis are the hallmark pathology findings. Tumorous swelling, eosinophilia, and obliterative phlebitis are other frequently observed features. Polyclonal elevations of serum IgG4 are found in most but not all patients. SUMMARY: IgG4-RSD is an underrecognized condition about which knowledge is now growing rapidly. Yet there remain many unknowns with regard to its cause, pathogenesis, various clinical presentations, approach to treatment, Disease monitoring, and long-term outcomes. A wide variety of organs can be involved in IgG4-RSD. Clinicians should be aware of this entity and consider the diagnosis in the appropriate settings.
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Treatment approaches to IgG4-related Systemic Disease
Current Opinion in Rheumatology, 2020Co-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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to report the first case of muscle specific tyrosine kinase musk antibody ab positive myasthenia gravis mg evolving into immunoglobulin ig g4 related Disease p3 134
Neurology, 2016Co-Authors: Pooja Raibagkar, John H. StoneAbstract:Objective: To report the first case of muscle specific tyrosine kinase (MuSK) antibody (ab) positive myasthenia gravis (MG) evolving into immunoglobulin (Ig) G4 related Disease. Background: IgG4 related Disease is an immune mediated, inflammatory condition most commonly affecting pancreas, salivary gland, lacrimal gland and retroperitoneum. It affects meninges and pituitary gland in central nervous system (NS) but involvement of peripheral NS is not known. MuSK ab is proven to be of IgG4 subclass and causes MG which differs from acetyline choline receptor (AChR) ab MG. Methods: Case report. Results: A 54 year old woman presented with fatigue and intermittent bulbar weakness. Three months later MG was diagnosed with negative AChR ab and positive MuSK ab. Treatment with intravenous Ig was initiated without benefit. She required hospitalization for myasthenic crisis three months later, manifested by dysphagia, dysarthria and dyspnea. She was treated with plasmapheresis with significant improvement. Prednisone 60mg/day was started. Mycophenolate mofetil was added a month later. Thymectomy was performed with histopathology showing extensive fatty replacement. She had poor control on prednisone and cellcept; requiring plasma exchange three times/week. A year and half later, she reported acute back pain. MRI of the spine demonstrated retroperitoneal and iliac lymphadenopathy. Open biopsy showed follicular hyperplasia with marked plasmacytosis and numerous IgG4 plasma cells. Serum IgG4 level was elevated at 525 mg/dl along with total Ig 2560, IgG1 1800 and IgG2 849. She met the diagnostic criteria for IgG4 related Systemic Disease involving lymph nodes. Conclusions: Our case is so far the first case showing potential clinical association of MuSK MG and Systemic IgG4 related Disease. This unique temporal relationship of medically refractory MuSK MG caused by IgG4 antibody to later development of IgG4 related Disease help further understand the unknown pathogenesis of IgG4 related Disease from the well understood autoimmune Disease, MG. Disclosure: Dr. Raibagkar has nothing to disclose. Dr. Stone has nothing to disclose.
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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, 2011Co-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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Eosinophilic angiocentric fibrosis is a form of IgG4-related Systemic Disease.
The American Journal of Surgical Pathology, 2011Co-Authors: Vikram Deshpande, Arezou Khosroshahi, Gunnlaugur P. Nielsen, Daniel L. Hamilos, John H. StoneAbstract:BackgroundEosinophilic angiocentric fibrosis (EAF) is an uncommon tumefactive lesion of the orbit and upper respiratory tract of unknown etiology. The condition is characterized histologically by concentric layers of fibrosis around small-caliber arteries and a mixed inflammatory infiltrate dominate
Arezou Khosroshahi - One of the best experts on this subject based on the ideXlab platform.
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A clinical overview of IgG4-related Systemic Disease.
Current Opinion in Rheumatology, 2020Co-Authors: Arezou Khosroshahi, John H. StoneAbstract:PURPOSE OF REVIEW: To 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 FINDINGS: The term IgG4-RSD encompasses a variety of clinical entities once regarded as being entirely separate Diseases. The list of organs associated with this condition is growing steadily. Tissue biopsies reveal striking histopathological similarity, regardless of which organ is involved, although subtle differences across organs exist. Diffuse lymphoplasmacytic infiltrates, presence of abundant IgG4-positive plasma cells and extensive fibrosis are the hallmark pathology findings. Tumorous swelling, eosinophilia, and obliterative phlebitis are other frequently observed features. Polyclonal elevations of serum IgG4 are found in most but not all patients. SUMMARY: IgG4-RSD is an underrecognized condition about which knowledge is now growing rapidly. Yet there remain many unknowns with regard to its cause, pathogenesis, various clinical presentations, approach to treatment, Disease monitoring, and long-term outcomes. A wide variety of organs can be involved in IgG4-RSD. Clinicians should be aware of this entity and consider the diagnosis in the appropriate settings.
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Treatment approaches to IgG4-related Systemic Disease
Current Opinion in Rheumatology, 2020Co-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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IgG4-related Systemic Disease as a Cause of “Idiopathic” Orbital Inflammation, Including Orbital Myositis, and Trigeminal Nerve Involvement
Survey of Ophthalmology, 2011Co-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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Eosinophilic angiocentric fibrosis is a form of IgG4-related Systemic Disease.
The American Journal of Surgical Pathology, 2011Co-Authors: Vikram Deshpande, Arezou Khosroshahi, Gunnlaugur P. Nielsen, Daniel L. Hamilos, John H. StoneAbstract:BackgroundEosinophilic angiocentric fibrosis (EAF) is an uncommon tumefactive lesion of the orbit and upper respiratory tract of unknown etiology. The condition is characterized histologically by concentric layers of fibrosis around small-caliber arteries and a mixed inflammatory infiltrate dominate
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subclassification of autoimmune pancreatitis a histologic classification with clinical significance
The American Journal of Surgical Pathology, 2011Co-Authors: Vikram Deshpande, Arezou Khosroshahi, John H. Stone, Rajib Gupta, Nisha I Sainani, Dushyant V Sahani, Renu K Virk, Cristina R Ferrone, Gregory Y LauwersAbstract:Autoimmune pancreatitis (AIP) is a chronic inflammatory Disease of the pancreas. Examination of pancreatic resection specimens from patients with AIP has shown that there are 2 subclasses of this Disease. However, there is no widely accepted pathologic classification scheme and the clinical significance of such a classification remains to be established. In this study, we revisited the subclassification of AIP and examine whether this provides clinically and prognostically meaningful information. We evaluated 29 pancreatic resection specimens from patients with AIP. Demographic, clinical, and imaging data were recorded, as was evidence of extrapancreatic manifestations. In addition to a detailed and semiquantitative histologic evaluation, immunohistochemistry for IgG4 was performed on pancreatic and extrapancreatic tissues. We also evaluated 48 consecutive cases of chronic pancreatitis, not otherwise specified. The resected specimens could readily be subclassified into 2 subtypes: type 1 (n = 11) and type 2 (n = 18). In comparison with patients with type 2 Disease, patients with type 1 Disease were significantly more likely to be males (P = 0.09), older (P = 0.02), and present with jaundice (P = 0.01), and less likely to be associated with abdominal pain (P = 0.04). On imaging, the pancreatic tail cut-off sign was exclusively seen in patients with type 2 Disease (4 of 10 cases). Hypercellular inflamed interlobular stroma was unique to type 1 pattern (91%), whereas significant ductal injury in the form of microabscesses and ductal ulceration was almost exclusively seen in type 2 pattern (78%). Eight of 10 patients with a type 1 pattern had evidence of a Systemic Disease. Three patients with type 2 Disease had recurrent episodes of pancreatitis after their pancreatic resection. In comparison with the cohort of chronic pancreatitis, not otherwise specified, type 2 AIP cases were less likely to be associated with a history of alcohol abuse, and showed significantly more foci of periductal inflammation and neutrophilic microabscesses. Our review of pancreatic resection specimens shows 2 histologically distinct forms of AIP. Our data support the concept that type 1 AIP is a Systemic Disease and is the pancreatic manifestation of IgG4-related Systemic Disease. Type 2 Disease is confined to the pancreas. The intensity of the periductal inflammatory infiltrate and the presence of ductal neutrophilic abscesses are features that assist in distinguishing type 2 AIP from chronic pancreatitis, not otherwise specified. Although imperfect, clinical and imaging features may help distinguish the 2 subtypes of AIP. On the basis of these significant differences between the 2 types of AIP, we advocate the position that all subsequent studies attempt to substratify their patients into these 2 groups.
Vikram Deshpande - 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, 2011Co-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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Eosinophilic angiocentric fibrosis is a form of IgG4-related Systemic Disease.
The American Journal of Surgical Pathology, 2011Co-Authors: Vikram Deshpande, Arezou Khosroshahi, Gunnlaugur P. Nielsen, Daniel L. Hamilos, John H. StoneAbstract:BackgroundEosinophilic angiocentric fibrosis (EAF) is an uncommon tumefactive lesion of the orbit and upper respiratory tract of unknown etiology. The condition is characterized histologically by concentric layers of fibrosis around small-caliber arteries and a mixed inflammatory infiltrate dominate
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subclassification of autoimmune pancreatitis a histologic classification with clinical significance
The American Journal of Surgical Pathology, 2011Co-Authors: Vikram Deshpande, Arezou Khosroshahi, John H. Stone, Rajib Gupta, Nisha I Sainani, Dushyant V Sahani, Renu K Virk, Cristina R Ferrone, Gregory Y LauwersAbstract:Autoimmune pancreatitis (AIP) is a chronic inflammatory Disease of the pancreas. Examination of pancreatic resection specimens from patients with AIP has shown that there are 2 subclasses of this Disease. However, there is no widely accepted pathologic classification scheme and the clinical significance of such a classification remains to be established. In this study, we revisited the subclassification of AIP and examine whether this provides clinically and prognostically meaningful information. We evaluated 29 pancreatic resection specimens from patients with AIP. Demographic, clinical, and imaging data were recorded, as was evidence of extrapancreatic manifestations. In addition to a detailed and semiquantitative histologic evaluation, immunohistochemistry for IgG4 was performed on pancreatic and extrapancreatic tissues. We also evaluated 48 consecutive cases of chronic pancreatitis, not otherwise specified. The resected specimens could readily be subclassified into 2 subtypes: type 1 (n = 11) and type 2 (n = 18). In comparison with patients with type 2 Disease, patients with type 1 Disease were significantly more likely to be males (P = 0.09), older (P = 0.02), and present with jaundice (P = 0.01), and less likely to be associated with abdominal pain (P = 0.04). On imaging, the pancreatic tail cut-off sign was exclusively seen in patients with type 2 Disease (4 of 10 cases). Hypercellular inflamed interlobular stroma was unique to type 1 pattern (91%), whereas significant ductal injury in the form of microabscesses and ductal ulceration was almost exclusively seen in type 2 pattern (78%). Eight of 10 patients with a type 1 pattern had evidence of a Systemic Disease. Three patients with type 2 Disease had recurrent episodes of pancreatitis after their pancreatic resection. In comparison with the cohort of chronic pancreatitis, not otherwise specified, type 2 AIP cases were less likely to be associated with a history of alcohol abuse, and showed significantly more foci of periductal inflammation and neutrophilic microabscesses. Our review of pancreatic resection specimens shows 2 histologically distinct forms of AIP. Our data support the concept that type 1 AIP is a Systemic Disease and is the pancreatic manifestation of IgG4-related Systemic Disease. Type 2 Disease is confined to the pancreas. The intensity of the periductal inflammatory infiltrate and the presence of ductal neutrophilic abscesses are features that assist in distinguishing type 2 AIP from chronic pancreatitis, not otherwise specified. Although imperfect, clinical and imaging features may help distinguish the 2 subtypes of AIP. On the basis of these significant differences between the 2 types of AIP, we advocate the position that all subsequent studies attempt to substratify their patients into these 2 groups.
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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.
Eunhee S. Yi - One of the best experts on this subject based on the ideXlab platform.
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Immunoglobulin G4-related Disease mimicking asthma.
Canadian Respiratory Journal, 2020Co-Authors: Hiroshi Sekiguchi, Ryohei Horie, Timothy R. Aksamit, Eunhee S. YiAbstract:Immunoglobulin (Ig) G4-related Disease (also known as ‘IgG4-related sclerosing Disease’, ‘IgG4-related Systemic Disease’ or ‘hyper-IgG4-Disease’) is a recently recognized Systemic fibroinflammatory Disease associated with IgG4-positive plasma cells in tissue lesions. IgG4-related Disease was initially described as autoimmune pancreatitis, but it is now known to affect virtually any organ. The authors describe a patient presenting with multi-organ manifestations, including airway inflammation mimicking asthma, pulmonary parenchymal infiltrates, intrathoracic lymphadenopathy, submandibular gland swelling and a kidney mass.
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pulmonary manifestations of immunoglobulin g4 related sclerosing Disease
European Respiratory Journal, 2012Co-Authors: Jay H Ryu, Hiroshi Sekiguchi, Eunhee S. YiAbstract:Immunoglobulin (Ig)G4-related sclerosing Disease (ISD) (also called IgG4-related Systemic Disease, IgG4-related Disease or hyper-IgG4 Disease) is a recently described Systemic fibroinflammatory Disease associated with elevated circulating levels of IgG4. Although initial descriptions of this disorder focused on its pancreatic presentation (autoimmune pancreatitis), it has become apparent that ISD is a Systemic Disease with many facets. The lesion of ISD is characterised by lymphoplasmacytic inflammation, fibrosis, phlebitis and increased numbers of IgG4-positive plasma cells. The Disease can either be localised to one or two organs, or be present with diffuse multi-organ Disease. Furthermore, lesions in different organs can present simultaneously or metachronously. In the thorax, lesions associated with ISD have been described in the lung parenchyma, airways and pleura, as well as the mediastinum. Data published to date suggest that ISD may account for a portion of various fibroinflammatory conditions of unknown cause encountered in the chest, including inflammatory pseudotumours, idiopathic interstitial pneumonias, fibrosing mediastinitis, inflammatory pleural lesions and, occasionally, airway Disease. Some aspects of pulmonary manifestations attributed to ISD remain controversial and additional studies are needed to clarify the relationship along with the increasing relevance of this disorder to pulmonary medicine.
Naoki Takahashi - One of the best experts on this subject based on the ideXlab platform.
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Autoimmune pancreatitis: an update
Abdominal Radiology, 2019Co-Authors: Ashish Khandelwal, Dai Inoue, Naoki TakahashiAbstract:Objective Since the time of inception of autoimmune pancreatitis (AIP), our knowledge of autoimmune pancreatitis has expanded significantly. The aim of this review is to provide an update on clinical manifestations, diagnosis, imaging features, and treatment of AIP. Background and clinical significance Type 1 AIP is the pancreatic manifestation of IgG4-related Systemic Disease, which can be diagnosed using a combination of clinical, histopathological, pancreatic imaging findings in conjunction with manifestation in other organs, as well of responsiveness to steroid treatment. It is vital to differentiate AIP from pancreatic cancer since both can mimic each other clinically and radiologically. Type 2 AIP is a rare but distinct subtype of AIP which occurs mostly in the younger patient. Conclusion AIP is steroid-responsive chronic pancreatitis with distinct manifestations on imaging.
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Autoimmune pancreatitis: an update.
Abdominal Radiology, 2019Co-Authors: Ashish Khandelwal, Dai Inoue, Naoki TakahashiAbstract:Since the time of inception of autoimmune pancreatitis (AIP), our knowledge of autoimmune pancreatitis has expanded significantly. The aim of this review is to provide an update on clinical manifestations, diagnosis, imaging features, and treatment of AIP. Type 1 AIP is the pancreatic manifestation of IgG4-related Systemic Disease, which can be diagnosed using a combination of clinical, histopathological, pancreatic imaging findings in conjunction with manifestation in other organs, as well of responsiveness to steroid treatment. It is vital to differentiate AIP from pancreatic cancer since both can mimic each other clinically and radiologically. Type 2 AIP is a rare but distinct subtype of AIP which occurs mostly in the younger patient. AIP is steroid-responsive chronic pancreatitis with distinct manifestations on imaging.
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Diagnosis of IgG4-related Tubulointerstitial Nephritis
Journal of The American Society of Nephrology, 2011Co-Authors: Yassaman Raissian, Thomas C. Smyrk, Samih H. Nasr, Christopher P. Larsen, Robert B. Colvin, Naoki Takahashi, Ami Bhalodia, Aliyah R. Sohani, Lizhi Zhang, Suresh T. ChariAbstract:IgG4-related Systemic Disease is an autoimmune Disease that was first recognized in the pancreas but also affects other organs. This Disease may manifest as tubulointerstitial nephritis (IgG4-TIN), but its clinicopathologic features in the kidney are not well described. Of the 35 patients with IgG4-TIN whose renal tissue specimens we examined, 27 (77%) had acute or progressive chronic renal failure, 29 (83%) had involvement of other organ systems, and 18 of 23 (78%) had radiographic abnormalities. Elevated total IgG or IgG4 serum levels were present in 79%. All pathologic specimens featured plasma cell–rich TIN, with most showing diffuse, expansile interstitial fibrosis. Immune complexes along the tubular basement membranes were present in 25 of 30 (83%). All specimens had a moderate to marked increase in IgG4+ plasma cells by immunohistochemistry. We used a control group of 175 pathologic specimens with plasma cell–rich interstitial infiltrates that can mimic IgG4-TIN to examine the diagnostic utility of IgG4 immunostaining. Excluding pauci-immune necrotizing and crescentic glomerulonephritis, IgG4 immunohistochemistry had a sensitivity of 100% (95% CI 90–100%) and a specificity of 92% (95% CI 86–95%) for IgG4-TIN. Of the 19 patients with renal failure for whom treatment and follow-up data were available, 17 (89%) responded to prednisone. In summary, because no single test definitively diagnoses IgG4-related Systemic Disease, we rely on a combination of histologic, immunophenotypic, clinical, radiographic, and laboratory features. When the Disease manifests in the kidney, our data support diagnostic criteria that can distinguish IgG4-TIN from other types of TIN.
