Chronic sclerosing sialadenitis is a chronic longlasting inflammatory condition affecting the salivary gland. Igg4 related systemic disease is now recognized as a systemic disease that may affect various organs. The contributing authors extensively discuss the topic from different perspectives, including the pathogenesis of conditions such as cellular senescence and innate immunity as well as diseases such as igg4 related sclerosing cholangitis and intraductal papillary neoplasm. Since 2001, when a possible link between igg4 and sclerosing lesions was identified, 1 idiopathic fibro. Suggested diagnostic criteria for igg4 related ht include. Increased numbers of igg4 expressing plasma cells and an increased ratio 30% of igg4 iggexpressing plasma cells have been suggested as possible criteria. Although initial descriptions of this disorder focused on its pancreatic presentation autoimmune pancreatitis, it has. Described initially as a form of sclerosing autoimmune pancreatitis now designated as type i autoimmune pancreatitis. Health, general autoimmune diseases causes of development and progression diagnosis b cells epidemiology histochemistry histology, pathological methods immunoglobulin g health aspects physiological aspects pancreatitis. Igg4 related sclerosing disease igg4 related systemic sclerosing disease.
Pathology of the bile duct yasuni nakanuma springer. Because there are not specific antibodies for this disease. Pathogenesis and clinical manifestations of igg4related disease. Provides extensive discussions on issues including recent emerging pathogenesis such as cellular senescence and innate immunity, and new diseases such as igg4 related sclerosing cholangitis and intraductal papillary neoplasm. Igg4related sclerosing cholangitis radiology reference. Vol 385 april 11, 2015 1461 rare in igg4related disease. The same policy has been also adopted in the consensus statement on the pathology of igg4 related disease 3. Igg4 related disease igg4 rd is a rare fibroinflammatory condition that can affect almost any organ, characterized by swollen lesions and often by eosinophilia and elevated serum igg4 concentrations. This condition has been known by many other names in the past, such as igg4 related sclerosing disease, igg4 related systemic disease, igg4 related systemic sclerosing disease, igg4 syndrome, and igg4 associated disease, however a consensus in 2012 agreed upon the use of igg4related disease.
Patients with igg4related sclerosing disease usually demonstrate a dramatic response to corticosteroid therapy, often within a few weeks, although spontaneous resolution may occur 3,29,30. Igg4 related disease is characterized by lymphoplasmacytic inflammation and fibrosis, often leading to massforming lesions in different organs. The syndrome affects predominantly middleaged and elderly patients, with male predominance. Igg4 related disease igg4 rd is a multiorgan system disease that has been recognized in the last 10 years. Igg4related disease igg4 rd, formerly known as igg4 related systemic disease, is a chronic inflammatory condition characterized by tissue infiltration with lymphocytes and igg4 secreting plasma cells, various degrees of fibrosis scarring and a usually prompt response to oral steroids. Lymphoplasmacytic sclerosing pancreatitis, a prototypal histological type of aip, is now recognized as a systemic igg4. The disease is characterized by dense bile duct infiltration by igg4 positive plasma cells resulting in extensive fibrosis. Identification of a novel antibody associated with autoimmune pancreatitis. This multiorgan immunemediated condition links many disorders previously regarded as isolated, singleorgan diseases without any known underlying systemic condition. Common features include igg4 related autoimmune pancreatitis, swelling of or within an organ system an inflammatory pseudotumor, salivary gland disease which can lead to enlargement of the salivary glands, swollen lymph nodes lymphadenopathy, skin manifestations, and symptoms consistent with allergies or asthma. The exact etiology of igg4related disease remains unclear with no known role of the igg4 molecule itself being identified.
The variable organ dysfunction reflects the clinical presentation. Igg4related sclerosing disease has been described in the orbit and. Igg4 ratio indicated sclerosing peritonitis was an igg4 related disease. Consequently, th2 pathways are no longer believed to be central to disease pathophysiology. Igg4related disease is a recently recognized systemic syndrome characterized by massforming lesions with lymphoplasmacytic infiltration, increase in the number of igg4. Igg4related systemic sclerosing disease of the ocular.
Igg4related disease is a multiorgan immunemediated chronic fibroinflammatory condition characterized by elevated serum igg4 concentrations, tumefaction, and tissue infiltration by igg4 positive plasma cells. People are often described as being generally well at the time of diagnosis, although some may give a history of. Igg4 related disease igg4rd statpearls ncbi bookshelf. When igg4 related disease affects the bile ducts, it is called igg4 related sclerosing cholangitis. Described initially as a form of sclerosing autoimmune pancreatitis now. We read with great interest the editorial by fox and fox1 describing the use of serum immunoglobulin g4 igg4 concentrations as a marker for igg4 related disease igg4 rd. Igg4related disease is a protean condition that mimics many malignant, infectious, and inflammatory disorders. Immunoglobulin igg4 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. It was first described as a systemic condition in 2003 when extrapancreatic manifestations were identified in patients with autoimmune. The histopathology of igg4related disease springerlink. The distinction between aip and pancreas cancer is vital, but is hard to make in some cases, for example in lesions with focal pancreatic enlargement andor negative serological tests. Presents a comprehensive overview of research on bile duct pathology, from basic understanding to practical aspects. However, a recent consensus statement on the pathology of igg4 rd 9 has recognized that a large number of conditions that are outside the igg4 rd spectrum. The criteria for a diagnosis of igg4 related sclerosing disease of the orbit have not been well established.
Igg4related disease has been described as an indolent condition. A novel clinical entity, igg4related disease igg4rd. Igg4related hashimotos thyroiditis of the thyroid gland. It is a chronic, systemic, inflammatory condition of unknown etiology. In approximately 5170% of people with this disease, serum igg4 concentrations are elevated during an. Atypical manifestations of igg4related sclerosing disease. Pubmed is a searchable database of medical literature and lists journal articles that discuss igg4related dacryoadenitis and sialadenitis. When igg4related disease is suspected, serum protein electrophoresis and igg. Few years later, igg4 positive cells were found in other organs and the term igg4 related systemic disease was suggested. The disease is so named because the antibody subtype igg4 can be detected on tissue samples and often at elevated levels in the. Diagnostic guidelines for igg4related disease with a focus on. Clinical manifestations are apparent in the pancreas, bile duct, gallbladder, salivary gland, retroperitoneum, kidney, lung and prostate, in which tissue fibrosis with obliterative phlebitis is pathologically induced table table1. The pathology is very specific and looks similar in all organs.
Igg4related sclerosing disease is a systemic disease characterized by extensive igg4 positive plasma cells and tlymphocyte infiltration of various organs. Pathology, jackson memorial hospital, holtz 2042c, 1611 nw 12th ave, miami, fl 336 email. Rare diagnosis of igg4 related systemic disease by lip. Idiopathic retroperitoneal fibrosis ormond disease. Increasing evidence suggests that igg4 rd is an autoimmune condition, much like rheumatoid arthritis and lupus. Requires additional clinical, serological or radiological evidence to confirm the diagnosis of igg4 related disease. Igg4 related sclerosing disease is a syndrome affecting various sites with elevated serum. Clinical manifestations are apparent in the pancreas, bile duct, gallbladder, salivary gland, retroperitoneum, kidney, lung and prostate, in which tissue fibrosis with obliterative. The contributing authors extensively discuss the topics from different perspectives, including the pathogenesis of conditions such as cellular senescence and innate immunity as well as diseases such as igg4 related sclerosing cholangitis and intraductal papillary neoplasm. Rd, a systemic fibroinflammatory condition that is characterized by mass lesions andor strictures with classical histopathological findings in involved organs. Igg4 associated disease is a relatively newly discovered entity characterized by sclerosing inflammation, a lymphoplasmacytic infiltration full of igg4 positive plasma cells and frequently associated with elevated serum igg4 concentrations. Biopsy diagnosis of type 1 autoimmune pancreatitis. This igg4related sclerosing cholangitis is the most common extrapancreatic manifestation of type 1 aip igg4related, present in over 70 percent of such patients 4. Immunoglobulin g4 igg4 related disease igg4 rd is an immunemediated condition that can affect almost any organ.
Patients with igg4 related sclerosing disease usually demonstrate a dramatic response to corticosteroid therapy, often within a few weeks, although spontaneous resolution may occur 3,29,30. Igg4related systemic sclerosing disease of the ocular adnexa. Igg4related disease igg4rd is known as an igg4related systemic disease, hyperigg4. Keywords igg4 related sclerosing disease igg4 related pachymeningitis nonvasculitic autoimmune in. Case 1 uterine adenosarcoma with sarcomatous overgrowth had the highest igg4 positivity and igg. Igg4related disease igg4 rd is a multiorgan fibroinflammatory condition of unknown aetiology that is defined by its unique histopathological features, that are fairly similar regardless of the affected organ. However, to our knowledge, there has been only one reported case of igg4 related sclerosing disease that man. Igg4related sclerosing cholangitis, also known as autoimmune cholangiopathy, is part of the spectrum of igg4 related disease but can also occur in isolation.
Sclerosing peritonitis associated with gynecological. Igg4related disease is an immunemediated condition, meaning that it involves the occurrence of disease in organs as the result of a dysregulated immune system. Although autoimmune pancreatitis is present in most patients with igg4related sclerosing disease, the latter has been reported in patients with no pancreatic involvement 20,28. Igg4 related chronic sclerosing sialadenitis patients may show an elevated serum igg4 levels. Endoscopic retrograde cholangiography and magnetic resonance cholangiography. This book is distributed under the terms of the creative commons attribution 4. It is a separate entity to primary sclerosing cholangitis pathology. Igg4 rd is a fascinating clinical entity including a wide variety of diseases, formerly diagnosed as mikuliczs disease, autoimmune pancreatitis aip, interstitial nephritis, prostatitis and retroperitoneal fibrosis.
Igg4related disease is a newlydescribed rare syndrome consisting of many disease entities. A 74yearold male complained of dysphagia and abdominal pain. Igg4 rd has a marked propensity to present as massforming lesions. An elevated serum titer of immunoglobulin g4 igg4, the least common 3% to 6% of the 4 subclasses of igg, is a surrogate marker for the recently characterized igg4 related sclerosing disease. It was recognised as a unified entity only 10 years ago. Seminars in diagnostic pathology the pathology of igg4 related. Igg4related sclerosing cholangitis a form of sclerosing cholangitis that is clinically distinct from primary sclerosing cholangitis may occur as part of the igg4rd. Discrepancies, if any, must be resolved in an interdisciplinary setting and with further evaluation, including a rebiopsy if necessary. Igg4related sclerosing disease is a systemic disease characterized by extensive igg4positive plasma cells and tlymphocyte infiltration of various organs. Igg4 related sclerosing sialadenitis a retrospective analysis. The purpose of this article is to present the pathologic and clinical features of igg4 related sclerosing cholangitis isc, illustrate the associated imaging findings, and discuss treatment of the disorder. Consensus statement on the pathology of igg4related disease. The classic igg4 rd patient is a middleaged to elderly individual.
Recognition and management of common, rare, and novel serum protein. Clinicopathologic analysis of igg4related skin disease. Click on the link to view a sample search on this topic. Although possibly based on opinion rather than on objective assessments, symptoms, if any, are commonly described as mild in the medical literature. Igg4 related disease is now considered a systemic disease that might affect every organ with progressively growing. Pseudotumor formation is the most common and characteristic clinical symptom. Igg4related dacryoadenitis and sialadenitis rare disease. The histopathology of igg4related disease was a specific focus of the. Sclerosing mesenteritis is a rare inflammatory and fibrosing disorder of unknown etiology, while igg4. Sc can mimic primary sclerosing cholangitis psc, cholangiocarcinoma cca, and pancreatic. Igg4related sclerosing disease, an emerging entity. Diagnostic guidelines for igg4related disease with a. Relatively rare in occurrence, this condition is benign, but presents as hard, indurated and enlarged masses that are clinically indistinguishable from salivary gland neoplasms or tumors.
Consensus statement on the pathology of igg4 related disease. Membranous glomerulopathy can be a manifestation of igg4 related renal disease. In our study on further follow up of 2436 months, none of the cases showed other manifestations of igg4 related systemic disease. Igg4related disease igg4rd is a systemic autoimmune fibroinflammatory. Igg4related sclerosing disease, also known as igg4 related systemic disease igg4 rsd, hyper igg4 disease and igg4 related disease is an autoimmune disease in which inflammatory cells cause fibrosis, the deposition of connective tissue, in one or more organs.
Relationship between rosaidorfman disease and igg4. Pulmonary manifestations of immunoglobulin g4related. Gastric mass gastritis and gastric ulcers can be associated with igg4 related sclerosing disease and aip 11, 12. American journal of clinical pathology, volume 7, issue 5, may 2012. Necrosis, discrete granulomata, and xanthogranulomatous changes are atypical and, when present, suggest other diagnoses. Orphanet is a european reference portal for information on rare diseases and orphan drugs. Masaki y, et al a novel clinical entity, igg4related disease igg4rd.
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