scholarly journals Thoracic involvement and imaging patterns in IgG4-related disease

2021 ◽  
Vol 30 (162) ◽  
pp. 210078
Author(s):  
Romain Muller ◽  
Paul Habert ◽  
Mikael Ebbo ◽  
Julie Graveleau ◽  
Mathieu Groh ◽  
...  

ObjectiveImmunoglobulin G4-related disease (IgG4-RD) is a rare orphan disease. Lung, pleura, pericardium, mediastinum, aorta and lymph node involvement has been reported with variable frequency and mostly in Asian studies. The objective of this study was to describe thoracic involvement assessed by high-resolution thoracic computed tomography (CT) in Caucasian patients with IgG4-RD.MethodsThoracic CT scans before treatment were retrospectively collected through the French case registry of IgG4-RD and a single tertiary referral centre. CT scans were reviewed by two experts in thoracic imagery blinded from clinical data.Results48 IgG4-RD patients with thoracic involvement were analysed. All had American College of Rheumatology/European League Against Rheumatism classification scores ≥20 and comprehensive diagnostic criteria for IgG4-RD. CT scan findings showed heterogeneous lesions. Seven patterns were observed: peribronchovascular involvement (56%), lymph node enlargement (31%), nodular disease (25%), interstitial disease (25%), ground-glass opacities (10%), pleural disease (8%) and retromediastinal fibrosis (4%). In 37% of cases two or more patterns were associated. Asthma was significantly associated with peribronchovascular involvement (p=0.04). Among eight patients evaluated by CT scan before and after treatments, only two patients with interstitial disease displayed no improvement.ConclusionThoracic involvement of IgG4-RD is heterogeneous and likely underestimated. The main thoracic CT scan patterns are peribronchovascular thickening and thoracic lymph nodes.

2015 ◽  
Vol 67 (4) ◽  
pp. 557-561 ◽  
Author(s):  
Adrian C Bateman ◽  
Margaret R Ashton-Key ◽  
Sanjay Jogai

2012 ◽  
Vol 2012 ◽  
pp. 1-8 ◽  
Author(s):  
Yasuharu Sato ◽  
Tadashi Yoshino

Lymphadenopathy is frequently observed in patients with immunoglobulin G4-related disease (IgG4-RD) and sometimes appears as the first manifestation of the disease. The diagnosis of IgG4-related lymphadenopathy is complicated owing to a great histological diversity, with at least 5 histological subtypes. Indeed, lymph node biopsy may be performed under the suspicion that the lymphadenopathy is a malignant lymphoma or other lymphoproliferative disorder. The diagnosis of IgG4-RD is characterized by both elevated serum IgG4 (>135?mg/dL) and histopathological features, including a dense lymphoplasmacytic infiltrate rich in IgG4+plasma cells (IgG4+/IgG+plasma cell ratio >40%). However, patients with hyper-interleukin (IL-) 6 syndromes such as multicentric Castleman’s disease, rheumatoid arthritis, and other immune-mediated conditions frequently show lymph node involvement and often fulfill the diagnostic criteria for IgG4-RD. Owing to these factors, IgG4-RD cannot be differentiated from hyper-IL-6 syndromes on the basis of histological findings alone. Laboratory analyses are crucial to differentiate between the 2 diseases. Hyper-IL-6 syndromes are characterized by elevated serum levels of IgG, IgA, IgM, and C-reactive protein (CRP); thrombocytosis; anemia; hypoalbuminemia; hypocholesterolemia. In contrast, IgG4-RD does not share any of these characteristics. Therefore, the diagnosis of IgG4-RD requires not only pathological findings but also clinical and laboratory analyses.


2013 ◽  
Vol 137 (9) ◽  
pp. 1282-1285 ◽  
Author(s):  
Kate E. Grimm ◽  
Antony Bakke ◽  
Dennis P. O'Malley

Context.—Immunoglobulin G4 (IgG4)–related disease is a recently described entity that presents as mass-forming lesions in soft tissue, exocrine glands, and in lymph nodes as IgG4-related lymphadenopathy. The underlying pathologic mechanism of IgG4-related disease is unclear; however, rituximab (an anti-CD20 monoclonal antibody) has been shown to have clinical efficacy. Objective.—To look for the presence or absence of CD20 on the IgG4-expressing plasma cells in IgG4-related lymphadenopathy. Design.—Twelve flow cytometry cases were identified through a retrospective review from the authors' institutions files. Cases were selected by the presence of a lymph node biopsy specimen with increased IgG4 plasma cells by immunohistochemistry and a histologic diagnosis compatible with IgG4-related lymphadenopathy. Results.—We report dim CD20 expression on plasma cells in all cases for which a plasma cell population was clearly identified by flow cytometry. These cases were from patients with lymph node biopsy specimens that met published criteria for IgG4-related lymphadenopathy. Conclusions.—This finding may be one potential explanation for the clinical efficacy of rituximab in IgG4-related disease.


1987 ◽  
Vol 28 (3) ◽  
pp. 263-269 ◽  
Author(s):  
S. P. Strijk

Ninety-one patients with non-Hodgkin lymphoma (NHL) were subjected to computed tomography (CT) and lymphography. Both examinations agreed in 74 patients (81%) with regard to the infradiaphragmatic lymph nodes. In patients undergoing CT prior to lymphography, the concordance amounted to 75 per cent. When lymphography was the initial examination, the concordance amounted to 86 per cent. Lymphography was abnormal in 30 per cent of the patients with a normal CT scan and in 93 per cent of those with an abnormal CT scan as the first examination. CT was abnormal in 4 per cent of patients with a normal lymphogram and in 84 per cent of those with an abnormal lymphogram as the first examination. CT did not detect mesenteric or retrocrural lymph node enlargement in the absence of retroperitoneal lymph node involvement. Eleven patients had extranodal manifestations of the disease (excluding liver and spleen), and 3 were detected primarily with CT. Lymphography is the most complete examination for the infradiaphragmatic lymph nodes for staging purposes. Although CT outlined the disease better, it changed the ***lymphographic diagnosis in only 2 per cent of the patients. Lymphography modified the CT stage in 15 per cent of the patients. When abdominal CT is performed first, in staging patients with NHL, lymphography will only yield additional information when CT is normal or equivocal.


2006 ◽  
Vol 13 (6) ◽  
pp. 311-316 ◽  
Author(s):  
Mark O Turner ◽  
John R Mayo ◽  
Nestor L Müller ◽  
Michael Schulzer ◽  
J Mark FitzGerald

BACKGROUND: Computed tomography (CT) scans are used extensively to investigate chest disease because of their cross-sectional perspective and superior contrast resolution compared with chest radiographs. These advantages lead to a more accurate imaging assessment of thoracic disease. The actual use and evaluation of the clinical impact of thoracic CT has not been assessed since scanners became widely available.OBJECTIVE: To identify patterns of utilization, waiting times and the impact of CT scan results on clinical diagnoses.DESIGN: A before and after survey of physicians who had ordered thoracic CT scans.SETTING: Vancouver General Hospital – a tertiary care teaching centre in Vancouver, British Columbia.SUBJECTS: Physicians who had ordered CT scans.INTERVENTION: Physicians completed a standard questionnaire before and after the CT scan result was available.MEASUREMENTS: Changes in the clinical diagnosis, estimates of the probabilities for the diagnosis both before and after the CT scan, and waiting times.RESULTS: Four hundred fifty-four thoracic CT cases had completed questionnaires, of whom 80% were outpatients. A change in diagnosis was made in 48% of cases (25% with a normal CT scan and 23% with CT scan findings that indicated a different diagnosis). The largest change in probability scores for the clinical diagnosis before and after the CT scan was 43.9% for normal scans, while it was 36.3% for a different diagnosis and 26.3% for the same diagnosis. High-priority scans were associated with decreased waiting time (−7.89 days for each unit increase in priority).CONCLUSIONS: The CT scan results were associated with a change in diagnosis in 48% of cases. Normal scans constituted 25% of the total and had the greatest impact scores. Waiting times were highly correlated with increased urgency of the presenting problem.


Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 33-34
Author(s):  
Luisa Fernanda Sánchez-Valledor ◽  
Thomas M. Habermann ◽  
Iván Murrieta-Álvarez ◽  
Andrés A. León-Peña ◽  
Yahveth Cantero-Fortiz ◽  
...  

Background: Hodgkin's lymphoma (HL) is the model of curative care with radiation therapy, combination chemotherapy, staging approaches, peripheral blood stem cell transplantation, and immunotherapy. However, the value of the novel anti cancer drugs has been recently analyzed and questioned in view of the results in the real improvement of overall survival (OS). Material and methods: All consecutive patients seeking medical care after 1986 in our institution as a result of HL and followed for at least 3 months were entered in the study. A diagnosis of HL was based on the histological study of a pathology specimen, mainly a lymph node; the same pathologist analyzed all the specimens and defined the histological subtype. Clinical stage was defined according to the Ann Arbor classification. Bone marrow biopsies were done only in patients with clinical stages III or IV. Computed tomography (CT) scans were done in all cases, prior to starting the treatment. Fluorodeoxyglucose positron emission tomography (FDG-PET) scans were performed since 2002. Between 1986 and 1997, patients were treated with MOPP, and after 1997 with ABVD as frontline therapy. For stages I and II, four cycles of chemotherapy were delivered and a computerized tomography (CT) scan was performed; if lymph node enlargement were present at this point in time, four additional cycles were given, whereas two additional cycles were given if the CT scan was negative. For stages III and IV, the CT scans were performed at the end of six cycles and two or four more cycles. Mediastinal radiotherapy was delivered only to persons with a positive FDG-PET scan at the end of the treatment. Patients showing activity after these treatments were considered as refractory and treated with four courses of ICE (ifosfamide, carboplatin and etoposide). Autologous or allogeneic peripheral blood hematopoietic stem cell transplants (HSCT) were given to refractory patients after achieving a complete remission (CR): High-dose melphalan (200mg/m2) was employed in autologous transplants, whereas cyclophosphamide, fludarabine and busulfan were employed in allogeneic transplants, all of them from HLA-identical siblings. After the completion of the treatment, patients were every two months for one year and every four months later on. No FDG-PET scans were done during the follow up, unless clinically indicated. Results: Among 91 patients with HL identified between 1986 and 2020, 88 were followed three months or more and were included in the analysis. There were 37 females and 51 males. The median age was 29years (range5-73years). There were 62 patients with nodular sclerosing HL (70%), 19 mixed cellularity (HL), 2 lymphocyte depleted HL, and one lymphocyte predominant HL; in 4 cases the histologic variant could not be defined. According to Ann Arbor classification, 5 patients were found in stage I, 48 in stage II, 19 in stage III and 16 in stage IV. Ten patients presented with a mediastinal mass larger than 10 cm. in the chest X-ray film. Three cases presented with relapsed disease. Patients have been followed for a median of 114 months (range4-402). 44 patients are alive, 10 have died and 34 were lost to follow-up. Median OS for all the patients has not been reached, being above 402 months, the OS at 310 months is 88% and at 402 months 77%. Median OS has not been reached and is above 94, 109, 90 and 98 months for stages I, II, III and IV, respectively (p=0.2). The 310-month OS was 83% for patients treated with MOPP and 88% for those treated with ABVD (HR:0.76, 95% CI 0.2-2.8; p=0.6).Sixteen patients (18%) were refractory to the treatment and 9 (10%) relapsed; they were treated with ICE followed by HSCT (autologous 15 patients and allogeneic 10 patients). Patients who underwent auto-HSCT had a median survival of 329.1 months and an OS of 92.3%, whereas those given allo-HSCT had a median survival of 59.3 months and an OS of 45.7% (HR0.2, 95%CI 0.04-1.3, p=0.057). The OS of patients given or not HSCT was 73.5% and 92.9% at 266 and 404 months, respectively (HR4.09, 95%CI 1.0-16.6, p=0.01). The OS was similar. The causes of death were breast carcinoma in 2 cases, liver carcinoma in one and uncontrolled lymphoma activity in the remaining. Conclusion: HL may be less aggressive in Mexican population than in Caucasians. Combined chemotherapy renders acceptable results, irrespective of clinical stage. Disclosures No relevant conflicts of interest to declare.


2014 ◽  
Author(s):  
Jiamin Liu ◽  
Jocelyn Zhao ◽  
Joanne Hoffman ◽  
Jianhua Yao ◽  
Weidong Zhang ◽  
...  

2020 ◽  
Vol 38 (6_suppl) ◽  
pp. 445-445
Author(s):  
Laura Marandino ◽  
Antonella Capozza ◽  
Alberto Briganti ◽  
Daniele Raggi ◽  
Elena Farè ◽  
...  

445 Background: FDG-PET/CT has limited utility in clinical N0 (cN0) patients (pts) with MIBC who receive neoadjuvant chemotherapy and RC or RC alone (Dason, AUA19). Methods: In PURE-01 (NCT02736266), 3 courses of 200 mg pembrolizumab, q3 weeks, were administered prior to RC. Pts were assessed with thorax-abdomen CT scan and with PET/CT scan during screening and before RC. Imaging review and analysis was internally performed. For each pt with lymph node (LN) increased uptake in abdomino-pelvic area, the SUVmax and the short-axis size of the most intense LN were recorded. All pts underwent extended pelvic LN dissection (LND) with packeted node submission. Results: From 02/17 to 06/2019, 103 total evaluable pts (206 PET/TC scans) were enrolled ad treated. Six pts (5.8%) had LN uptake at baseline PET/CT: mean SUVmax=2.75; mean short axis: 6.2 mm. Eight pts (7.8%) had LN uptake at PET/CT post-pembrolizumab: mean SUVmax=4.21; mean short axis: 7.2mm. The rate of pathologic LN positive (pN+) disease was 15.5% (16 pts). The performance of post-pembrolizumab PET/CT in predicting pN+ disease is indicated in the Table. In total, 4/6 pts (66.7%) with baseline FDG uptake revealed as pN+ vs 12/97 (12.4%) with no baseline FDG uptakes (p=0.005). A total of 39 pts (37.9%) developed inflammatory FDG-uptakes post-pembrolizumab in several target organs/regions: top 5 sites were thyroid (N=21, 61.8%), stomach and mediastinum (13 pts each, 12.6%), lung (N=10, 9.7%), other lymph nodes (N=4, 3.9%). These changes were clinically evident (signs/symptoms or laboratory changes) in 15 pts (38.5%). Conclusions: Criteria for eligibility of cN0 pts to single-agent neoadjuvant pembrolizumab trials may be enhanced with PET/CT use. Three cycles of pembrolizumab determined profound inflammatory changes, whose long-term impact on safety is still to be determined. Clinical trial information: NCT02736266 . [Table: see text]


2005 ◽  
Vol 173 (4S) ◽  
pp. 432-432
Author(s):  
Georg C. Bartsch ◽  
Norbert Blumstein ◽  
Ludwig J. Rinnab ◽  
Richard E. Hautmann ◽  
Peter M. Messer ◽  
...  

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