scholarly journals Cross-sectional pictorial review of IgG4-related disease

2019 ◽  
Vol 92 (1103) ◽  
pp. 20190448 ◽  
Author(s):  
Darya Kurowecki ◽  
Michael N. Patlas ◽  
Ehsan A. Haider ◽  
Abdullah Alabousi
Keyword(s):  
Plasma Cells ◽  
Multidetector Ct ◽  
Current Knowledge ◽  
Systemic Disease ◽  
Autoimmune Disorder ◽  
Immunoglobulin G4 ◽  
Cross Sectional ◽  
Related Disease ◽  
Pictorial Review ◽  
Serum Igg4

Immunoglobulin G4-related disease (IgG4-RD) is an autoimmune disorder characterized by the infiltration of one or more organs with IgG4-positive plasma cells resulting in inflammatory lesions and fibrosis. Although the pancreas is the most commonly affected organ, involvement of extrapancreatic organs is an increasingly recognized manifestation of the disease. Patients may be asymptomatic and serum IgG4 concentrations may be elevated or normal. Treatment consists of glucocorticosteroid treatment, with excellent response. A definitive diagnosis requires histopathology with imaging playing a key role in avoiding treatment delays. This pictorial review will focus on the most current knowledge regarding IgG4-RD including its common and less common manifestations and the roles of multidetector CT, MRI and ultrasound in the diagnosis and management of suspected IgG4-RD. Knowledge of the varied imaging findings of this multi systemic disease is essential for radiologists to avoid misdiagnosis and assist with timely and effective treatment.

scholarly journals An Overlapping Case of IgG4-Related Disease and Systemic Lupus Erythematosus

2019 ◽  
Vol 7 ◽  
pp. 232470961986229 ◽  
Author(s):  
Srikanth Naramala ◽  
Sharmi Biswas ◽  
Sreedhar Adapa ◽  
Vijay Gayam ◽  
Venu Madhav Konala ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Systemic Disease ◽  
Lymph Node Biopsy ◽  
Systemic Lupus ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Double Stranded Dna ◽  
Serum Igg4

We are reporting a case of a 63-year-old Chinese female who presented to the rheumatology clinic with positive antinuclear antibody and unintentional weight loss along with lymphadenopathy. Further workup revealed eosinophilia, elevated anti–double stranded DNA, serum protein, and serum IgG4 (immunoglobulin G4). The patient was diagnosed with systemic lupus erythematosus. Due to the raised IgG4 level along with eosinophilia and diffuse lymphadenopathy, IgG4-related systemic disease was suspected. It was confirmed with IgG4 staining on lymph node biopsy. Our case is presenting the fact that systemic lupus erythematosus and IgG4-related disease can be present in the same patient with multiple overlapping features making accurate diagnosis challenging.

scholarly journals Immunoglobulin G4 Sclerosing Cholangitis: An Unusual Cause of Obstructive Jaundice—Case Report and Literature Review

2018 ◽  
Vol 2018 ◽  
pp. 1-5
Author(s):  
Pragya Shrestha ◽  
Brian Le ◽  
Brent Wagner ◽  
William Pompella ◽  
Paras Karmacharya
Keyword(s):  
Sclerosing Cholangitis ◽  
Plasma Cells ◽  
Systemic Disease ◽  
Clinical Manifestations ◽  
Disease Process ◽  
High Serum ◽  
Diagnostic Challenge ◽  
Obliterative Phlebitis ◽  
Immunoglobulin G4 ◽  
Serum Igg4

IgG4-related sclerosing cholangitis (IgG4-SC) is one of the most common extra-pancreatic manifestation of IgG4-related disease (IgG4-RD) and is clinically distinct from primary sclerosing cholangitis (PSC). IgG4-RD is an increasingly recognized immune-mediated fibroinflammatory systemic disease, mostly affecting middle-aged and older male populations that can affect multiple organs. The presence of extra-biliary clinical manifestations of IgG4-RD, such as parotid and lacrimal swelling, lymphadenopathy, autoimmune pancreatitis, and retroperitoneal fibrosis, if present could provide important clues to diagnosis. High serum IgG4 levels, characteristic radiological (e.g., sausage-shaped pancreas or periaortitis) or biopsy findings (high percentage of IgG4+ plasma cells, lymphoplasmacytic infiltrate, storiform fibrosis, or obliterative phlebitis) in the setting of these features is diagnostic of this disease process. However, isolated IgG4-SC might be a diagnostic challenge, and the distinction is important as management of this disorder is vastly different from other causes of cholangitis such as PSC. Systemic corticosteroid therapy is the mainstay of therapy.

scholarly journals Fevers, Sweats, Weight Loss, Oh My! When IgG4 Related Disease Comes Knocking. . .

2021 ◽  
Vol 14 ◽  
pp. 117954762110463
Author(s):  
Francis Essien ◽  
Graey Wolfley ◽  
Joshua Tate ◽  
Matthew Carroll
Keyword(s):  
Weight Loss ◽  
Plasma Cells ◽  
Systemic Disease ◽  
Elevated Serum ◽  
Acute Onset ◽  
Immunoglobulin G4 ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Rare Manifestation ◽  
Organ Manifestations

Immunoglobulin G4-related systemic disease (IgG4-RSD) is a fibro-inflammatory immune condition characterized by IgG4 positive plasma cells, fibrosis, and frequently elevated serum IgG4 level. Akin to sarcoidosis, IgG4-RSD is a systemic disease with diverse organ manifestations linked by common histopathologic features. IgG4-RSD typically presents sub acutely without significant constitutional symptoms or fever. Hepatic Inflammatory pseudotumor (HIP) is a rare manifestation of IgG4-RSD composed of dense lymphoplasmacytic infiltrate and extensive fibrosis. We present an older Asian male who presented with acute onset of fever and weight loss found to have IgG4-RSD complicated by a HIP and concomitant MSSA abscess.

scholarly journals IgG4 Related Disease of The Larynx Mimicking Malignancy: A Case Report and Review of the Literature

2021 ◽  
Author(s):  
Sevda Akyol ◽  
Ozlem Saraydaroglu ◽  
Omer Afsin Ozmen
Keyword(s):  
Plasma Cells ◽  
Clinical Laboratory ◽  
Review Of The Literature ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Storiform Fibrosis ◽  
Lymphoplasmacytic Infiltration ◽  
Igg4 Level

Abstract Objectives: Immunoglobulin G4–related disease is characterized by increased serum IgG4 level, enlargement in the relevant organs and histopathologically intense storiform fibrosis, lymphoplasmacytic infiltration rich in IgG4 positive plasma cells, and obliterative phlebitis.Methods and Results: In this report, a patient who underwent a laryngeal biopsy with a pre-diagnosis of malignancy, but had findings consistent with immunoglobulin G4–related disease in the biopsy sample, is described.Conclusion: Immunoglobulin G4–related disease can be seen in very rare localizations. It should be kept in mind in differential diagnosis when tissues especially containing inflammation rich in plasma cells are encountered. Clinical, laboratory and pathological correlation is extremely important in the diagnosis of an IgG4-related disease.

scholarly journals Primary Sclerosing Cholangitis Associated with Elevated Immunoglobulin-G4: A Preliminary Study

2012 ◽  
Vol 2012 ◽  
pp. 1-4 ◽  
Author(s):  
Baran Parhizkar ◽  
Amir Houshang Mohammad Alizadeh ◽  
Hamid Asadzadeh Aghdaee ◽  
Habib Malekpour ◽  
Amir Hossein Entezari
Keyword(s):  
Esophageal Varices ◽  
Systemic Disease ◽  
Cross Sectional Study ◽  
Chi Square ◽  
Immunoglobulin G4 ◽  
Cross Sectional ◽  
Serum Igg4 ◽  
Inflammatory Bowel ◽  
Spss Software ◽  
Igg4 Level

Background. Immunoglobulin IgG4-associated cholangitis (IAC) disease is a systemic disease histologically characterized by extensive T lymphocytes and IgG4 positive plasma cell infiltration in various organs. Prevalence of IAC in PSC patients was reported to be between 7% and 11.6% in a few previous studies. This study was carried out to evaluate frequency of serum IgG4 level in PSC patient referred to the gastroenterology ward of Taleghani educational hospital in Tehran, Iran. Material and Methods. This study was a prospective analytical cross-sectional study. Clinical presentation, laboratory values, imaging changes, inflammatory bowel disease (IBD), esophageal varices, ascites, and child score in newly PSC patients with elevated IgG4 were determined and compared with PSC patients with normal levels of IgG4. Data was analyzed by using SPSS software. The frequency and standard deviations were calculated. Differences among groups were evaluated by using the chi-square, fisher exact, and Mann-Whitney U tests. Results. 34 patients with PSC were examined in the study period, of which 9 cases (26.5%) had high IgG4 levels. Most of the patients were male, 23 cases (67.6%) and nonsmoker, 26 cases (76.5%). Patient average age was 47 years old (range 21–67 years). There was not any significant relationship among patients with IAC and PSC patients in terms of variables such as age, smoking, presence of IBD, ascites, esophageal varices, child score, and imaging findings (). Conclusion. IAC should be suspected in cases of unexplained biliary strictures with increased serum IgG4. Testing PSC patients for IgG4 and treating those who have high levels with corticosteroids in clinical trials should be considered in future studies.

scholarly journals Multiple Artery Aneurysms: Unusual Presentation of IgG4 Vasculopathy

2021 ◽  
Vol 11 ◽  
pp. 17
Author(s):  
Anurag Yadav ◽  
Geetha Godasu ◽  
Tarvinder Bir Singh Buxi ◽  
Swapnil Sheth
Keyword(s):  
Plasma Cells ◽  
Systemic Disease ◽  
Artery Aneurysm ◽  
High Serum ◽  
Immunoglobulin G4 ◽  
Atypical Chest Pain ◽  
Igg4 Related Disease ◽  
Target Organs ◽  
Serum Igg4 ◽  
Giant Coronary Artery Aneurysm

Immunoglobulin G4 (IgG4)-related disease is a chronic systemic disease. It is characterized by inflammatory fibrosis and high serum IgG4 levels. IgG4-positive plasma cells infiltrate target organs in this disease. It may involve the pancreas, biliary tract, lacrimal glands, salivary glands, orbits, thyroid, kidneys, lymph nodes, or retroperitoneum. It may present as vasculitis with involvement of large to medium sized vessels such as the aorta, the common iliac, carotid, and coronary arteries. We present a case of 55-year-old male patient who presented with shortness of breath on exertion and atypical chest pain. On CT angiography, a giant coronary artery aneurysm involving the left anterior descending artery, multiple visceral and intercostal artery aneurysms, and nodular paravertebral soft-tissue thickening secondary to IgG4 vasculopathy.

scholarly journals Immunoglobulin G4-Related Disease Mimicking Asthma

2013 ◽  
Vol 20 (2) ◽  
pp. 87-89 ◽  
Author(s):  
Hiroshi Sekiguchi ◽  
Ryohei Horie ◽  
Timothy R Aksamit ◽  
Eunhee S Yi ◽  
Jay H Ryu
Keyword(s):  
Airway Inflammation ◽  
Submandibular Gland ◽  
Autoimmune Pancreatitis ◽  
Plasma Cells ◽  
Systemic Disease ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Organ Manifestations

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.

scholarly journals Immunoglobulin G4-related disease of the cavernous sinus with orbit invasion – A case report

2021 ◽  
Vol 12 ◽  
pp. 557
Author(s):  
Julie Mayeku ◽  
Jeremy Deisch ◽  
Miguel Angel Lopez-Gonzalez
Keyword(s):  
Cavernous Sinus ◽  
Plasma Cells ◽  
Surgical Intervention ◽  
Systemic Disease ◽  
Delayed Diagnosis ◽  
Unknown Etiology ◽  
Pathology Report ◽  
Partial Recovery ◽  
Immunoglobulin G4 ◽  
Related Disease

Background: Immunoglobulin G4-related disease (IgG4-RD) is a rare systemic disease of unknown etiology. It is characterized by tissue infiltration caused by IgG4 plasma cells and sclerosing inflammation of various body organs. At present, there are very few reported cases of IgG4-RD invasion of cavernous sinus and the orbit. Case Description: A 56-year-old female with a history of rheumatoid arthritis was presented with a gradual onset of right oculomotor, abducens, and trigeminal nerve deficits. Four weeks after the onset of symptoms, the patient developed gradual visual deficit. Following this, a trial of steroids was administered to the patient. However, the treatment did not work as expected and patient’s condition worsened. She progressed on to suffer complete visual loss in the right eye. Extensive work-up conducted on her turned out to be nondiagnostic. After this, the patient was referred to us for our evaluation. Neuroimaging revealed a right-sided cavernous sinus and orbital apex lesion. Given the lack of diagnosis and response to steroid treatment, we recommended surgical intervention and performed a modified pterional and pretemporal approach with extradural anterior clinoidectomy and transcavernous approach. We performed a lesion biopsy and cavernous sinus decompression, which helped in the partial recovery of visual function. The pathology report was consistent with IgG4-RD. Conclusion: IgG4-RD is a rare disease that occurs even less in combination with cavernous sinus and orbit invasion. The rarity of the disease and the diverse presentation of symptoms have sometimes caused delayed diagnosis and intervention. Patients who failed to respond to conservative management and patients in the fibrotic stage of the disease without other organ involvement may benefit from surgical intervention if amenable. Early suspicion, diagnosis, and intervention can facilitate better prognosis.

scholarly journals IgG4 Related Disease of Epididymis, Mimicking Testicular Malignancy – A Rare Entity

2019 ◽  
pp. 1-4
Author(s):  
LEELA KRISHNA ◽  
SRIRAM KRISHNAMOORTHY ◽  
HARIHARASUDHAN SEKAR ◽  
SUSRUTHAN MURALI ◽  
RAJENDIRAN SWAMINATHAN ◽  
...  
Keyword(s):  
Plasma Cells ◽  
Elevated Serum ◽  
Rare Entity ◽  
Left Testis ◽  
Immunoglobulin G4 ◽  
Systemic Involvement ◽  
Related Disease ◽  
Left Testicle ◽  
Serum Igg4 ◽  
Spindle Cell Proliferation

Immunoglobulin G4 related disease (IgG4-RD) is a systemic fibro inflammatory condition that usually presents with multiorgan involvement. We present a rare case of 54 year old male with an isolated IgG4-RD of epididymis. The patient presented with a progressive swelling of the left testicle. A clinical diagnosis of tuberculosis was made. Ultrasound scrotum showed a relatively hetero-echoic mass lesion involving the left epididymis in close proximity to the left testis. There was a focal spindle cell proliferation and an increase in number of plasma cells and keloid like collagen. Immunohistochemistry was positive for vimentin and IgG4 and negative for CD34. Serum level of IgG4 was elevated (165 mg per dL). Computed tomography of abdomen and thorax did not show any systemic involvement. HE was posted for excision of the epididymal mass. Intraoperatively, the mass was found to be densely adherent to left testicle and inseparable from it, necessitating left total orchiectomy. Histopathology and immunohistochemistry with elevated serum IgG4 levels confirmed the diagnosis of IgG4-RD of the epididymis. To the best of our knowledge, this condition is an extremely rare entity, with only very few cases of isolated IgG4-RD of epididymis reported in medical literature, with no other systemic manifestations.

scholarly journals Severe IgG4-Related Disease in a Young Child: A Diagnosis Challenge

2015 ◽  
Vol 2015 ◽  
pp. 1-4
Author(s):  
Susana Corujeira ◽  
Catarina Ferraz ◽  
Teresa Nunes ◽  
Elsa Fonseca ◽  
Luísa Guedes Vaz
Keyword(s):  
Plasma Cells ◽  
Elevated Serum ◽  
Female Child ◽  
Symptomatic Improvement ◽  
Organ Involvement ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Lymphoplasmacytic Infiltrate ◽  
Serum Igg4 ◽  
Tract Infections

Immunoglobulin G4-related disease (IgG4-RD) is an increasingly recognized syndrome that can appear with multiple organ involvement, typically with tumor-like swelling, lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, and elevated serum IgG4 concentrations. We report the case of a 22-month-old female child with failure to thrive and recurrent respiratory tract infections since 8 months of age. Physical examination was normal except for pulmonary auscultation with bilateral crackles and wheezes. Laboratory tests revealed elevated erythrocyte sedimentation rate, and elevated serum IgG and IgG4 with polyclonal hypergammaglobulinemia. Thoracic CT and MRI showed multiple mediastinal lymphadenopathies and a nodular posterior mediastinal mass in right paratracheal location with bronchial compression. Initial fine needle aspiration biopsy was compatible with reactive lymphadenopathy but after clinical worsening a thoracoscopic partial resection of the mass was performed and tissue biopsy revealed lymphoplasmacytic infiltrate and increased number of IgG4-positive plasma cells and a ratio of IgG4/IgG positive cells above 40%. Glucocorticoids therapy was started with symptomatic improvement, reduction in the size of the mass, and decrease of serum IgG4 levels after 6 weeks. There are very few reports of IgG4-RD in children. Long-term follow-up is necessary to monitor relapses and additional organ involvement.

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