scholarly journals IgG4-related Disease: a diagnostic challenge

2021 ◽  
Vol 11 ◽  
pp. e2021312
Author(s):  
Rodrigo Díaz Olmos ◽  
Marcelo Arlindo Vasconcelos Miranda Rodrigues ◽  
Cristiane Rúbia Ferreira ◽  
Rita de Cássia Franco Etrusco ◽  
Carla Romagnolli
2018 ◽  
Vol 32 (1) ◽  
pp. 29-35 ◽  
Author(s):  
Shahine Goulam-Houssein ◽  
Jeffrey L Grenville ◽  
Katerina Mastrocostas ◽  
David G Munoz ◽  
Amy Lin ◽  
...  

IgG4-related disease (IgG4-RD) is a multi-organ chronic inflammatory process caused by infiltration of IgG4-positive plasma cells in one or more organs. Intracranial involvement has only recently become better recognized. Our case series adds to the growing literature on the varying presentations of intracranial IgG4 by describing the clinical and imaging findings of three patients who presented to our institution with intracranial involvement. Our first patient presented with a mass-forming IgG4 pachymeningitis mimicking a sphenoid wing meningioma, which is to our knowledge the largest mass-forming pachymeningitis published in the literature. Our second case depicts another presentation of extensive IgG4 pachymeningitis involving both cavernous sinuses and surrounding Meckel’s caves. The third case describes a patient with presumed lymphocytic hypophysitis, which was later determined to be IgG4-related hypophysitis with concomitant pachymeningitis and perineural spread along the optic nerves. The delayed diagnoses in our cases illustrates the diagnostic challenge that clinicians face in differentiating intracranial IgG4-RD from other infiltrative diseases such as sarcoidosis, granulomatous disease, tuberculosis and lymphoma. Earlier consideration of IgG4-related hypophysitis and hypertrophic pachymeningitis in the differential diagnosis can prevent significant morbidity including unnecessary surgical intervention and organ failure secondary to extensive fibrosis.


Author(s):  
Monika Kukulska ◽  
Izabela Smoła ◽  
Agnieszka Haloń ◽  
Aleksander Stal

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Bolong Liu ◽  
Yong Huang ◽  
Luying Tang ◽  
Jiexia Guan ◽  
Xiangfu Zhou ◽  
...  

Abstract Background With widespread clinical application of imaging techniques, renal space-occupying lesions have been identified at an increasing frequency. Here, we report two rare cases, Castleman disease (CD) and IgG4-related disease (IgG4-RD), presenting primarily with the symptoms and imaging findings of kidney malignancy. Case presentation In case 1, an occupying lesion located in the right renal pelvis was detected using magnetic resonance imaging in a 32-year-old female who presented with hematuria and lumbago. First misdiagnosed as carcinoma of the renal pelvis, the patient underwent right radical nephroureterectomy. However, postoperative pathological and immunohistochemistry studies finally confirmed the diagnosis of CD. In case 2, a 45-year-old male presented with the chief complaint of anuria. Nephrostomy and renal biopsy indicated lymphoma, following which, antegrade urography and computed tomography urography were performed, which revealed bilateral hydronephrosis and mass lesions around the renal pelvis. Partial resection of the masses and frozen section examination indicated the diagnosis of CD. However, the results of postoperative histopathology and immunohistochemistry combined with serum IgG4 were consistent with IgG4-RD. Both the patients recovered well after drug treatment without recurrence of the diseases. Conclusions Inflammatory pseudotumor of CD and IgG4-RD with kidney involvement are primarily diagnosed by postoperative histopathology and can pose a preoperative diagnostic challenge because these lesions can masquerade as kidney malignancy. Therefore, we recommend core biopsy as a nonnegligible procedure to evaluate renal masses and potentially prevent unnecessary surgical treatment.


Author(s):  
Krishna Ramanathan ◽  
Satish Nair ◽  
V. Pavithra ◽  
Savith Kumar ◽  
Gurucharan Adoor ◽  
...  

Pathology ◽  
2020 ◽  
Vol 52 (3) ◽  
pp. 390-392
Author(s):  
Archana Pandita ◽  
Janice Wong

2019 ◽  
Vol 129 (7-8) ◽  
pp. 539-541
Author(s):  
Anouk Le Goueff ◽  
Frederic Vandergheynst ◽  
David Jayne

2021 ◽  
Author(s):  
Bolong Liu ◽  
Yong Huang ◽  
Luying Tang ◽  
Jiexia Guan ◽  
Xiangfu Zhou ◽  
...  

Abstract Background: With widespread clinical application of imaging techniques, renal space-occupying lesions have been identified at an increasing frequency. Here, we report two rare cases, Castleman disease (CD) and IgG4-related disease (IgG4-RD), presenting primarily with the symptoms and imaging findings of kidney malignancy.Case presentation: In case 1, an occupying lesion located in the right renal pelvis was detected using magnetic resonance imaging in a 32-year-old female who presented with hematuria and lumbago. First misdiagnosed as carcinoma of the renal pelvis, the patient underwent right radical nephroureterectomy. However, postoperative pathological and immunohistochemistry studies finally confirmed the diagnosis of CD. In case 2, a 45-year-old male presented with the chief complaint of anuria. Nephrostomy and renal biopsy indicated lymphoma, following which, antegrade urography and computed tomography urography were performed, which revealed bilateral hydronephrosis and mass lesions around the renal pelvis. Partial resection of the masses and frozen section examination indicated the diagnosis of CD. However, the results of postoperative histopathology and immunohistochemistry combined with serum IgG4 were consistent with IgG4-RD. Both the patients recovered well after drug treatment without recurrence of the diseases.Conclusions: Inflammatory pseudotumor of CD and IgG4-RD with kidney involvement are primarily diagnosed by postoperative histopathology and can pose a preoperative diagnostic challenge because these lesions can masquerade as kidney malignancy. Although rare, we recommend that clinicians consider CD and IgG4-RD when diagnosing patients with renal masses to avoid unnecessary surgical treatment.


2016 ◽  
Vol 7 (1) ◽  
pp. 108-111 ◽  
Author(s):  
Rumana Hussain ◽  
Abdul El-Khyat ◽  
Antonella Berry-Brincat

A 48-year-old lorry driver presented with 3 weeks of blurred vision, pain and diplopia. There was a right upper lid ptosis with some restriction of eye movements. A CT revealed an enlarged lacrimal gland and lacrimal gland biopsy showed IgG4-positive plasma cells. The patient responded to oral prednisolone and fully recovered. As a condition which mimics a number of diseases, an IgG4-related disease presents a diagnostic challenge and ought to be considered in both acute and chronic presentations.


2020 ◽  
Author(s):  
L Schulte ◽  
F Arnold ◽  
F Siegel ◽  
J Backhus ◽  
L Perkhofer ◽  
...  

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