Manifestation Of IgG4-related Disease Research Articles

A case of PLA2R-positive membranous nephropathy with subsequent development of IgG4-related disease.

Membranous nephropathy (MN) is a common cause of adult-onset nephrotic syndrome. It is also known as a minor but established renal manifestation of Immunoglobulin G4-related disease (IgG4-RD). Previous reports suggest that MN can also be an initial manifestation of IgG4-RD, all of which are phospholipase A2 receptor (PLA2R)-negative MN. We describe a case of PLA2R-positive MN that subsequently developed other manifestations of IgG4-RD. A 60-year-old male with nephrotic syndrome was diagnosed as primary MN with positive staining for PLA2R on the initial renal biopsy, which remained in partial remission with supportive therapy using angiotensin II receptor blocker (ARB) without steroid. About 1year later, a renal mass was detected during an annual checkup, and contrast-enhanced computed tomography revealed low-density masses in bilateral kidneys and the head of the pancreas. The findings of endoscopic biopsy of the pancreatic mass were consistent with autoimmune pancreatitis (AIP) and the second renal biopsy showed the findings of MN with tubulointerstitial nephritis, both of which led to a diagnosis of IgG4-RD. The second renal biopsy also showed positive PLA2R. The patient received oral glucocorticoid therapy for IgG4-RD, which improved IgG4-related AIP and renal masses and also resulted in complete remission of MN. To our knowledge, this is the first reported case of PLA2R-positive MN with subsequent development of IgG4-RD. It is sometimes difficult to determine whether PLA2R-positive MN occurring with IgG4-RD is primary MN or secondary MN associated with IgG4-RD. The possibility of developing IgG4-RD should be considered even when preceding MN is PLA2R-positive, suggesting of primary MN.

Papulonodular Lesions in Photo Exposed Areas as First Manifestation of IgG4-Related Disease

IgG4-related disease (IgG4-RD) or IgG4 syndrome is a chronic inflammatory condition of presumed autoimmune etiology, affecting mainly men in their sixth decade of life. It has a good prognosis following instituted treatment and is characterized by infiltration of IgG4 + plasma cells, predominantly in the pancreas, bile duct, lymph nodes, kidneys, retroperitoneum, lungs and salivary, parotid, submandibular and lacrimal glands. Skin manifestations of IgG4 syndrome are uncommon but should be known to the dermatologist. This report details arare case of IgG4 syndrome involving cutaneous and lymph nodes. A 53-year-old woman described a 15-year history of erythematous and pruritic papulo-nodular lesions, mostly in photoexposed areas of the face, neck and upper limbs. She explained that the lesions evolved with ulceration, healing and residual dyschromia. Furthermore, she reported relapsing lymphadenomegaly 2 years ago, primarily in the cervical, axillary and inguinal chains.

IgG4-Rich Lesions Associated With Intranasal Drug Use Can Mimic IgG4-Related Disease.

Manifestations of immunoglobulin G4-related disease (IgG4-RD) occur in several organ systems and anatomic locations, including the nasal cavity and paranasal sinuses. Other processes affecting the sinonasal tract, such as chronic rhinosinusitis, aspirin-exacerbated respiratory disease, and nasal polyposis, also involve IgG4. To characterize an association between IgG4 and nasal lesions arising in the clinical context of intranasal drug use. The cases of 3 patients (2 with histories of intranasal cocaine abuse, and 1 with intranasal heroin abuse) were evaluated. Clinical features of each case were compiled from the electronic medical record. Histologic morphology of surgical specimens was examined. Immunohistochemical staining was performed to assess involvement of/association with IgG4. Clinical features of these lesions included diffuse necrotic fibrinous debris, scarring, and endoscopically evident inflammation. Tissue sections showed acutely and chronically inflamed respiratory-type mucosa with abundant IgG4-positive plasma cells. Although these cases share some aspects in common with IgG4-RD, other definitive characteristics are absent, and notable differences exist. This series provides the first demonstration of increased IgG4 expression in nasal lesions associated with intranasal drug use. Despite some similarities, the pathologic processes and IgG4-rich infiltrates in these 3 cases seem to represent a different phenomenon that is not IgG4-RD. Although these lesions contain abundant IgG4-positive cells, they should not be mistaken for or conflated with IgG4-RD.

The "great imitator": IgG4-related disease of the oral cavity. Two case reports and scoping review.

This study aimed to review the lesser-known intraoral manifestations of immunoglobulin G4-related disease (IgG4-RD). In this paper we report an unprecedented case of oral IgG4-RD mimicking angiolymphoid hyperplasia with eosinophilia (ALHE), and another case presenting as plasma cell gingivitis. We then performed a scoping review of published cases of IgG4-RD involving the oral cavity. The following data were collected for each case: age, sex, intraoral site(s) involved, clinical appearance, imaging features, serum IgG4 values, histopathology, treatment, and follow-up duration. Fifty-one cases of oral IgG4-RD were published in literature. The hard palate and jaw bones were the two main locations reported, while the histological identification of a IgG4/IgG plasma cells ratio ≥40% was fundamental for diagnosis. Conversely, the pathological features of storiform fibrosis and obliterative phlebitis were not common. Future reports regarding oral IgG4-RD should report clear adherence to the recognized international diagnostic criteria of the disease.

Eosinophilic Gastroenteritis with Ascites, Elevated Serum IgG4, and Hypereosinophilic Syndrome: A Manifestation of IgG4-related Disease?

A 76-year-old woman with persistent diarrhea was referred to our hospital. She had purpura, peripheral eosinophilia (18,177/μL), and an elevated serum IgG4 level (819 mg/dL). Abdominal computed tomography revealed massive ascites and bowel edema. A skin biopsy of the purpura revealed leukocytoclastic vasculitis with prominent eosinophilic infiltration. Biopsies of the gastrointestinal mucosa revealed dense eosinophilic infiltration, indicating eosinophilic gastroenteritis (EG) associated with the hypereosinophilic syndrome. The number of IgG4-positive cells increased in the duodenal mucosa; however, the diagnostic criteria for IgG4-related disease (IgG4-RD) were not met. Whether or not EG with ascites is a manifestation of IgG4-RD warrants further investigation.

Coronary manifestations of immunoglobulin G4-related disease.

Coronary manifestations of immunoglobulin G4-related disease.

Intraconal Tumor-like mass as first manifestation of IgG4-related disease

Intraconal Tumor-like mass as first manifestation of IgG4-related disease

Thoracoabdominal manifestations of immunoglobulin G4-related disease

Immunoglobulin G4-related disease (IgG4-RD) comprises a group of immune-mediated diseases that, until recently, were considered separate entities. These entities have a similar clinical presentation, serological findings, and pathogenesis, so they are currently considered a single multisystemic disease. The common characteristic is the infiltration of involved tissues by plasma cells and lymphocytes that are positive for IgG4. Three major criteria have been proposed for the diagnosis of IgG4-RD: clinical, laboratory, and histological.The pancreas is the organ that is most affected by IgG4-RD, which can simulate a tumor. In this respect, a series of signs could help us suspect that the pancreatic findings do not reflect a tumor (halo sign, duct-penetrating sign, absence of vascular invasion, etc.). The differential diagnosis is important to avoid unnecessary surgical interventions.

Lymph Nodes With Increased IgG4-positive Plasma Cells and Patterns Suspicious for IgG4-related Disease: Can Lymph Nodes Be the Only Site of Disease?

Lymphadenopathy with increased immunoglobulin (Ig) G4 + plasma cells can be a nonspecific finding or a manifestation of immunoglobulin G4-related disease (IgG4-RD). It remains unclear whether there are characteristic pathologic features of IgG4-RD involving lymph nodes, or if IgG4-RD lymphadenopathy can occur without other manifestations of IgG4-RD. In this study, we assessed 55 lymph node biopsy specimens (44 men and 11 women with a mean age of 55y) with increased IgG4 + plasma cells that had 1 of the 6 well-described pathologic patterns. We also correlated these findings with IgG4 serum levels and followed these patients for 7 to 108 months (mean, 34.9mo) for the occurrence of extranodal IgG4-RD. We further compared lymphadenopathy in patients who developed other manifestations of IgG4-RD (RD + , n=20, 36%) versus those who did not (RD - , n=35, 64%). We found that there were only minor significant differences between 2 groups, including frequency of receiving treatment (RD + , 90% vs. RD - , 60%, P =0.021) and higher serum levels of C-reactive protein (>8mg/L, RD + , 53% vs. RD - , 13%, P =0.007). Other differences were either borderline or not significant, including mean age (RD + , 59.8y vs. RD - , 51.9y, P =0.097), male-to-female ratio (RD + , 16:4 vs. RD - , 28:7, P =1), constitutional symptoms (RD + , 25% vs. RD - , 9%, P =0.096), multiple enlarged lymph nodes (RD + , 45% vs. RD - , 26%, P =0.143), good response to therapy (RD + , 94% vs. RD - , 94%, P =1); higher serum IgG4 levels (>280mg/dL, RD + , 75% vs. RD - , 51%, P =0.086), anemia (RD + , 45% vs. RD - , 43%, P =0.877), leukopenia (RD + , 0% vs. RD - , 3%, P =0.446), thrombocytopenia (RD + , 10% vs. RD - , 6%, P =0.556), positivity for antinuclear antibody (RD + , 24% vs. RD - , 29%, P =0.688), elevated serum levels of lactate dehydrogenase (>225U/L, RD + , 0% vs. RD - , 20%, P =0.064), elevated serum IgE level (>100IU/mL, RD + , 75% vs. RD - , 92%, P =0.238), and hypergammaglobulinemia (RD + , 90% vs. RD - , 86%, P =0.754). There were also no differences in morphologic patterns ( P =0.466), IgG4 + cell location ( P =0.104), eosinophil counts (RD + , 10.3±11.3 vs. RD - , 13.4±17.5, P =0.496), Epstein-Barr virus positivity (RD + , 35% vs. RD - , 60%, P =0.074), and Epstein-Barr virus-positive cell location ( P =0.351). Our findings suggest that there are minimal differences between stringently defined IgG4-RD lymphadenopathy with versus without other manifestations of IgG4-RD. These findings also suggest the existence of IgG4-RD lymphadenopathy as the sole presentation of IgG4-RD.

S4 Autoimmune Pancreatitis Secondary to Immune Checkpoint Inhibitor Therapy (Type 3 AIP): Insights into a New Disease from Clinical Review and Serial Pancreatic Imaging

Introduction: Autoimmune pancreatitis (AIP), types 1 and 2, are well described. While type 1 AIP is a pancreatic manifestation of immunoglobulin G4-related disease (IgG4-RD) and type 2 AIP is a duct-centric pancreatic injury sometimes associated with inflammatory bowel disease. A third form of AIP (type 3) is caused by increased immune activity by immune checkpoint inhibitor therapy (ICI-℞) for advanced malignancies. We describe the clinico-radiological spectrum and short-term natural history of type 3 AIP. Methods: We performed a detailed clinical record review of the 248/11,165 (2.2%) adult patients receiving ICI-℞ who developed type 3 AIP (>3-fold serum lipase elevation + pain, absent other etiologies). A radiologist reviewed 379 abdominal computerized tomography scans (CT) and measured pancreas volume in a subset of CTs done before, during and after pancreatitis. Results: At onset, type 3 AIP was painful in 96 (38%) and asymptomatic in 154 (62%); none had jaundice or local/systemic complications. CT showed normal pancreas (43%), peri-pancreatic edema (27%), loss of feathery pattern (21%) or diffuse/focal pancreatic enlargement (10%). A minority achieved complete and persistent biochemical resolution, more often when ICI-℞ was held (32/89) vs no intervention (13/84, P = 0.003) or vs steroid treatment alone (2/23, P = 0.01). Early or late relapses occurred equally frequently regardless of intervention. Compared to pancreas volume before ICI-℞, >20% volume loss occurred in 55% 1 year post-pancreatitis, more so with dual ICI-Rx, regardless of pain or steroid therapy at presentation. 18% had new diabetes within 2 years of pancreatitis. No patient developed calcifications or duct dilation. Conclusion: Type 3 AIP is a novel drug-induced chronic inflammatory disease of the pancreas. Predominantly asymptomatic and mild in severity, it causes rapid pancreatic atrophy and functional endocrine failure despite steroid therapy or ICI-℞ withdrawal. Table 1. - Clinical profile of all type 3 and subjects in imaging cohorts Type 3 AIP* (n=248) Imaging cohort (n=93) Symptomatic (n=94) Asymptomatic (n=154) ≥3x lipase elevation cohort (n=48) 2x lipase elevation cohort (n=24) Normal lipase cohort (n=21) Median Age in years (IQR) 61 (50-68) 62 (54-69) 58 (49-67) 63 (54-69) 59 (55-67) Male N (%) 37 (39%) 72 (47%) 17 (35%) 18 (75%) 13 (62%) Cancer type (%) Genitourinary 27 (29%) 63 (41%) 20 (42%) 14 (58%) 10 (48%) Melanoma 26 (28%) 28 (18%) 16 (33%) 3 (13%) 5 (24%) Other 41 (44%) 63 (41%) 12 (25%) 7 (29%) 6 (29%) ICI Agent (%) CTLA-4 8 (9%) 10 (6%) 5 (10%) 2 (8%) 4 (19%) PD-1 62 (66%) 95 (62%) 28 (58%) 14 (58%) 10 (48%) Combined 24 (26%) 49 (32%) 15 (31%) 8 (33%) 7 (33%) ICI doses prior to elevated lipase (IQR) 4 (2-6) 3 (2-8) 5.5 (3-10) 4.5 (3-13) 3 (2-5, to normal lipase) Median days from ICI to pancreatitis (IQR) 114 (69-264) 113 (52-230) 124 (83-261) 175 (63-348) 95 (47-167, to normal lipase) ICI discontinued (%) 67 (71%) 74 (48%) 28 (58%) 14 (58%) 11 (52%) Risk factors (%) Alcohol consumption 40 (43%) 69 (45%) 22 (46%) 13 (54%) 12 (57%) Smoking history 52 (55%) 77 (50%) 24 (50%) 17 (71%) 7 (33%) Diabetes prior to pancreatitis 16 (17%) 44 (29%) 15 (31%) 6 (25%) 4 (19%) Drug allergy 56 (60%) 81 (53%) 22 (46%) 10 (42%) 10 (48%) Prior history of pancreatitis 5 (5%) 6 (4%) 2 (4%) 1 (4%) 0 *Autoimmune pancreatitis, IQR= interquartile range, ICI= immune checkpoint inhibitor, CTLA-4 (cytotoxic T lymphocyte-associated protein 4), PD-1 (programmed cell death receptor-1) and PD-L1 (programmed cell death ligand-1) Figure 1.: a: Serial pancreatic volume in normal lipase, 2x and 3x groups (median, cm3), b: Serial Pancreatic Volume: Single Agent vs Combination in 3x group (median, cm3), c: Percentage of patients with >20% pancreas volume loss in each group.

IgG4-positive plasma cells in nonspecific sialadenitis and sialolithiasis

AbstractChronic sclerosing sialadenitis is commonly regarded as a manifestation of IgG4-related disease. We previously found that a high IgG4 expression or IgG4-related disease could accompany nonspecific sialadenitis, whereas chronic sclerosing sialadenitis was not directly associated with IgG4-related disease. Our previous findings lead us to hypothesize that these inflammatory conditions of the submandibular gland signify a continuous progression of disease rather than different disease entities. We, therefore, aimed to determine the presence of IgG4-positivity and genuine IgG4-related disease in a cohort of 165 submandibular gland specimens from patients who underwent surgery due to chronic nonspecific sialadenitis or sialolithiasis. To do so, we re-evaluated histopathological features and divided samples into three groups: (A) nonspecific sialadenitis without known sialolithiasis, (B) sialadenitis with sialolithiasis, and (C) sialolithiasis without sialadenitis. We performed immunohistochemical staining for IgG4, IgG, and CD31, and assessed the Boston consensus statement criteria for IgG4-related disease in IgG4-positive samples. We also reviewed patient records and supplemented follow-up data with a questionnaire among patients with IgG4-positive samples. IgG4-positive plasma cells (range 1–344) were found in 131 samples. Among these, 19 samples were classified as IgG4-positive (≥70 IgG4-positive plasma cells/high-power field). Two IgG4-positive samples were histologically highly suggestive of IgG4-related disease, but only one had a clinically confirmed diagnosis of IgG4-related disease. Our results indicate that patients with sialadenitis and sialolithiasis often present with IgG4-positive lymphoplasmacytic infiltrates, but exceedingly rarely present with genuine IgG4-related disease. In sialolithiasis without sialadenitis, IgG4-positive plasma cells are often absent or appear in small numbers. These results support our hypothesis of a continuum of disease, and indicate that progressive inflammation of the submandibular gland leads to the development of more specific pathological features over time.

Severe asthma as the initial clinical manifestation of IgG4-related disease: a retrospective clinical study

BackgroundRespiratory involvement is common in immunoglobulin G4-related disease (IgG4-RD). However, severe asthma as the initial clinical manifestation of IgG4-RD is rare and might be neglected by respiratory clinicians. We aimed to explore the clinical characteristics and prognoses of patients with immunoglobulin G4-related disease (IgG4-RD) manifesting as severe asthma.MethodsA retrospective analysis of the clinical characteristics and prognoses of patients with severe asthma who were eventually diagnosed with IgG4-RD was performed in the Peking Union Medical College Hospital from 2013 to 2019.ResultsTwelve patients (5males, 7 females) were included. The mean age at enrollment and age of asthma onset were 59.4 ± 10.1 and 53.8 ± 10.4 years, respectively. The mean duration of asthma symptoms was 5.7 ± 2.0 years. In all patients, the proportion (25.1 ± 10.3%) and count (2.0 ± 1.1) × 109/L of eosinophils in peripheral blood increased. Additionally, all patients exhibited elevated total immunoglobulin E [IgE, (1279.3 ± 1257.9) KU/L] and IgG4 (9155.8 ± 9247.6) mg/dL. Bronchial wall thickening (n = 11) and mediastinal/hilar lymphadenopathy (n = 11) were major chest CT manifestations. All were pathologically diagnosed through surgical biopsy; submandibular gland (n = 8), supraclavicular lymph node (n = 2), stomach (n = 1), rashes (n = 1), lacrimal gland (n = 1) and thoracoscopic lung (n = 1) biopsies were performed. Asthma was well controlled by oral glucocorticoids (GCs), but some patients relapsed during tapering (n = 11). The refractory condition was controlled after increasing the dosage of GCs and add-on immunosuppressants.ConclusionsFor patients with middle age-onset severe asthma with elevated eosinophils, total IgE and IgG4 levels and available salivary gland ultrasound imaging, ruling out IgG4-RD is recommended. GCs used in combination with immunosuppressants is recommended to prevent relapse.

Isolated IgG4-related cholecystitis with localized gallbladder wall thickening mimicking gallbladder cancer: a case report and literature review

BackgroundIgG4-related cholecystitis, which is a manifestation of IgG4-related disease in the gallbladder, is associated with autoimmune pancreatitis or IgG4-related sclerosing cholangitis in most cases; isolated gallbladder lesions without systemic manifestations are very rare. Gallbladder wall thickening is often diffuse, but sometimes localized, in which case, differentiation from gallbladder cancer becomes difficult. The characteristic features of IgG4-related cholecystitis on imaging that would enable differentiation from gallbladder cancer remain poorly described.Case presentationWe present a rare case of isolated IgG4-related cholecystitis with localized gallbladder wall thickening that was clinically difficult to distinguish from malignancy before resection. An 82-year-old man was referred to our hospital because of gallbladder wall thickening on abdominal ultrasonography without any symptoms. Dynamic computed tomography of the abdomen showed localized wall thickening from the body to the fundus of the gallbladder that was enhanced from an early stage with a prolonged contrast effect. There were no other findings, such as pancreatic enlargement and bile duct dilatation. Magnetic resonance cholangiopancreatography revealed neither dilatation nor stenosis of the bile duct and pancreatic duct. Endoscopic ultrasonography (EUS) showed a smooth layered thickening of the gallbladder wall with a maximum thickness of 6 mm and a well-preserved outermost hyperechoic layer in the same area. Laparoscopic cholecystectomy was performed because malignancy could not be completely ruled out. Pathological examination of a resected specimen revealed IgG4-positive plasma cell infiltration, fibrosis, and phlebitis. Although the serum IgG4 level measured after resection was normal, the condition was ultimately diagnosed as probable IgG4-related cholecystitis according to the 2020 revised comprehensive diagnostic criteria for IgG4-related disease. The EUS images reflected the pathological findings, in which lymphocytic infiltration was distributed in a laminar fashion in the gallbladder wall.ConclusionsAlthough rare, isolated IgG4-related cholecystitis with localized wall thickening mimicking gallbladder cancer remains a clinical problem. A smooth laminar thickening of the gallbladder wall on EUS imaging could be one of the most informative characteristics for differentiating IgG4-related cholecystitis from gallbladder cancer.

Asymptomatic, reddish-brown papules and plaques in an elderly Japanese woman

Asymptomatic, reddish-brown papules and plaques in an elderly Japanese woman

\u201cStomach mass\u201d as the first manifestation of IgG4-related disease: a case report

BackgroundIgG4-related disease mainly manifests as organomegaly and is accompanied by tissue fibrosis (Mimori, Mod Rheumatol 29(2):213, 2019) which is frequently confused with tumour (Dawei et al., J Gastroenterol Hepatol 29(12):1375–8, 2020). There are few reports with of IgG4-related disease with the first clinical manifestation involving the stomach.Case presentationWe present the case of 46-year-old male patient with a “stomach tumour” as the first manifestation of IgG4-related disease. Gastroscopy showed a mass in the stomach, however, the pathology result was chronic inflammation with IgG4 positivity. CT scans of abdomen showed that the stomach wall was thick, the head of the pancreas was swollen, and retroperitoneal fibrosis was severe.The serum IgG4 level was 75 g/L (normal range 0.03–2.01 g/L).After treatment with methylprednisolone for one month, the symptoms were greatly relieved.ConclusionsTo reduce the suffering of patients and relieve their financial burden, we should consider the possibility of IgG4-related disease when the initial manifestation is a stomach mass.

IgG4-related kidney disease

IgG4-related disease (IgG4-RD) is an immune-mediated fibro-inflammatory disease characterized by the formation of sclerotic tumor-like masses with dense lymphoplasmacytic infiltrates containing a significant number of IgG4-plasma cells. IgG4-RD is characterized by a wide range of clinical manifestations since the disease can involve almost any organ. Renal da­mage is observed in about 15 % of patients with IgG4-RD. The article presents a review of data about clinical manifestations of IgG4-related kidney disease (IgG4-RKD), specific features of diagnosis, and modern treatment approaches. The review of scientific publications was conducted in the international electronic scientometric database PubMed using keywords “IgG4-related kidney disease”, “IgG4-RKD radiographic findings”, “IgG4-related tubulointerstitial nephritis”, “IgG4-related membranous glomerulonephritis” over the period 2012–2020. The most common renal lesions in IgG4-RD are tubulointerstitial nephritis and membranous glomerulonephritis. They occur predominantly in older males. At the time of diagnosis, patients mostly already have concomitant extrarenal manifestations of IgG4-RD (sialadenitis, pancreatitis, lymphadenopathy). The clinical picture is usually vague, although nephrotic syndrome and chronic renal failure may occur. Typical manifestations of IgG4-RKD on CT are multiple low-dense foci, thickening of the renal pelvis, diffuse kidney enlargement. Renal involvement in IgG4-RD is often accompanied by low levels of complement. Early detection and treatment of IgG4-RKD are extremely important due to the increase in morbidity and mortality due to the development of chronic kidney disease. Glucocorticoids (GC) are first-line drugs for IgG4-RD. Despite a good response to GC, even a slight delay in treatment can lead to the development of renal tissue atrophy. The use of immunosuppressive drugs is advisable in cases when the dose of GC cannot be reduced due to the constantly high activity of the di­sease. Rituximab (a CD20+ B-lymphocyte inhibitor) may be used in patients with a recurrent course of IgG4-RKD.

Gallbladder wall thickening in patients with IgG4-related diseases, with special emphasis on IgG4-related cholecystitis

Objectives Gallbladder (GB) wall thickening sometimes occurs in patients with autoimmune pancreatitis (AIP), a condition for which the name, IgG4-related cholecystitis, was proposed. We examined the radiological findings of the GB in patients with IgG4-related diseases and clinical features of patients with GB wall thickening and presented a hypothesis of its pathogenesis. Materials and methods GB wall thickening was defined by thickness ≥ 4 mm. GB wall thickness was examined in 258 patients with IgG4-related disease. Clinical and imaging findings of 200 patients with AIP with and without GB wall thickening were then compared. Results GB wall thickening was detected in 58 patients (29%) with AIP and two patients with isolated IgG4-related sclerosing cholangitis. In the 60 GBs examined, wall thickening was diffuse, with the walls possessing a smooth inner surface. No GB wall thickening was detected among the 56 patients with IgG4-related disease without AIP or IgG4-related sclerosing cholangitis. Bile duct stenosis was detected in 56 patients (97%) with AIP with GB wall thickening. Intraductal ultrasonography indicated cystic duct wall thickening connected to bile duct wall thickening in 11 of 14 (79%) patients with AIP or IgG4-related sclerosing cholangitis with GB wall thickening. Forty-eight patients in whom IgG4-related cholecystitis was diagnosed experienced resolution of the GB wall thickening after receiving steroid therapy. Conclusions Most cases of GB wall thickening in IgG4-related diseases are closely associated with IgG4-related sclerosing cholangitis and may be a manifestation of IgG4-related disease throughout the biliary tract, including the bile duct, cystic duct, and GB.

Pseudotumoral Auto-Immun Pancreatitis with Multiple Pancreatic Pseudo-Cysts: A Diagnosis Pitfall

Introduction: The autoimmune pancreatitis (AIP) is a chronic inflammatory disease secondary to autoimmune disorders. Its s considered as a manifestation of IgG4 related disease. Case Report: We report an exceptional case of AIP of a patient who presented with nonspecific symptoms leading to an MRI-diagnosis of a pancreatic tale tumor with strong presumption of cystadenocarcinoma rather than adenocarcinoma. Histopathological study revealed a tense lymphoplasmacytic infiltrate associated with storiform fibrosis and collagenization. Furthermore; many pseudocysts were associated lesions immunohistochemical tests revealed a diffuse staining of plasma cells by IgG and IgG4 antibodies. The diagnosis of an AIP with multiple pseudocysts mimicking a pancreatic tale tumor was made. Conclusion: These, all criterions considered together made our case, a rare entity that may be a challenging diagnosis leading sometimes, as the current case, to a massive surgery

18F-FDG PET/CT Manifestations of IgG4-related Disease.

Immunoglobulin G4-related disease (IgG4-RD) was not recognised as a systemic condition until 2003, when extra pancreatic manifestations were identified in patients with autoimmune pancreatitis. Since then, IgG4-RD has been described to involve virtually every organ system. It is highly responsive to immunosuppressants but can have detrimental effects if left untreated. Early recognition of the disease is, therefore, critical. The diagnosis of IgG4-RD is frequently challenging owing to its non-specific clinical manifestations, indolent nature and broad differential diagnoses. Although histopathological examination remains the cornerstone of diagnosis, imaging plays an important role in establishing extent of disease and identifying areas suitable for biopsy. 18F-Fluorodeoxyglucose (18F-FDG) positron emission tomography/computed tomography (PET/CT) has been demonstrated to be useful in assessing organ involvement, guiding biopsy and monitoring disease response. The 18F-FDG PET/CT scan is highly sensitive and able to evaluate multiorgan involvement in a single examination, a key advantage over conventional imaging modalities. A potential pitfall is its low specificity. As such, detailed knowledge of the imaging findings in IgG4-related disease is required to avoid misdiagnosis. This pictorial review aims to depict the diverse spectrum of imaging findings of IgG4-RD and the key imaging features to distinguish it from other important differential diagnoses.

Manifestations of IgG4-related disease in the head and neck

IgG4-related disease (IgG4-RD) is an immune-mediated disease condition that can affect almost any organ, including the head and neck. It is a chronic, systemic inflammation of unknown etiology. Tumor formation is the most common clinical symptom. Any tumor in the head and neck area is always a concern for an otorhinolaryngologist, head and neck surgeon, that we are dealing with malignant neoplastic growth. Ultrasound imaging, computer tomography, magnetic resonance imaging and even fine needle aspiration biopsy usually do not exclude neoplastic hyperplasia. Only open biopsy or excision biopsy followed by histopathological examination suggest the diagnosis of IgG4-RD, which requires further diagnosis, mainly serological. The authors present the most common IgG4-RD in the head and neck area, diagnostic criteria and their differentiation from apparently similar diseases.