scholarly journals Coincident relapsing polychondritis and IgG4–related disease: a diagnostic challenge

2019 ◽  
Vol 129 (7-8) ◽  
pp. 539-541
Author(s):  
Anouk Le Goueff ◽  
Frederic Vandergheynst ◽  
David Jayne
Keyword(s):  
Relapsing Polychondritis ◽  
Diagnostic Challenge ◽  
Related Disease ◽  
Igg4 Related Disease

scholarly journals IgG4-related intracranial disease

2018 ◽  
Vol 32 (1) ◽  
pp. 29-35 ◽  
Author(s):  
Shahine Goulam-Houssein ◽  
Jeffrey L Grenville ◽  
Katerina Mastrocostas ◽  
David G Munoz ◽  
Amy Lin ◽  
...  
Keyword(s):  
Plasma Cells ◽  
Surgical Intervention ◽  
Case Series ◽  
Hypertrophic Pachymeningitis ◽  
Diagnostic Challenge ◽  
Perineural Spread ◽  
Related Disease ◽  
The Third ◽  
Igg4 Related Disease ◽  
Intracranial Involvement

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.

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
Keyword(s):  
Diagnostic Challenge ◽  
Related Disease ◽  
Igg4 Related Disease

scholarly journals Autoimmune hepatitis and IgG4 related disease: a diagnostic challenge

2020 ◽  
Author(s):  
Monika Kukulska ◽  
Izabela Smoła ◽  
Agnieszka Haloń ◽  
Aleksander Stal
Keyword(s):  
Autoimmune Hepatitis ◽  
Diagnostic Challenge ◽  
Related Disease ◽  
Igg4 Related Disease

scholarly journals Inflammatory pseudotumor of Castleman disease and IgG4-related disease masquerading as kidney malignancy

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Bolong Liu ◽  
Yong Huang ◽  
Luying Tang ◽  
Jiexia Guan ◽  
Xiangfu Zhou ◽  
...  
Keyword(s):  
Renal Pelvis ◽  
Inflammatory Pseudotumor ◽  
Frozen Section ◽  
Imaging Techniques ◽  
Castleman Disease ◽  
Diagnostic Challenge ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Kidney Involvement ◽  
Serum Igg4

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.

Igg4 Related Disease of Skull Base—A Diagnostic Challenge

2020 ◽  
Author(s):  
Krishna Ramanathan ◽  
Satish Nair ◽  
V. Pavithra ◽  
Savith Kumar ◽  
Gurucharan Adoor ◽  
...  
Keyword(s):  
Skull Base ◽  
Diagnostic Challenge ◽  
Related Disease ◽  
Igg4 Related Disease

AB1038 INFLAMMATORY ORBITAL DISEASES: THE EXPERIENCE OF A TERTIARY RHEUMATOLOGY CENTRE

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1810-1811
Author(s):  
M. S. Aksun ◽  
T. K. Sahin ◽  
E. C. Bolek ◽  
L. Kiliç ◽  
E. G. Bulut ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Vision Loss ◽  
Clinical Laboratory ◽  
Wide Spectrum ◽  
Age At Onset ◽  
Relapsing Polychondritis ◽  
Inflammatory Lesions ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Orbital Disease

Background:Inflammatory lesions of orbital disease encompass a wide spectrum of clinical entities including rheumatic disorders.Objectives:To describe our experiences in adult patients who applied to a tertiary rheumatology center due to orbital disease.Methods:This is a retrospective descriptive study and data were extracted from patient’ charts. We described the clinical, laboratory, radiologic, histopathological presentations and final diagnoses of patients with inflammatory orbital disease who applied to our rheumatology clinic between January 2014 and December 2019.Results:Thirty-eight patients (Female: 63.2%) were identified; median age at onset of orbital symptoms was 44.5 (min.-max 5-72) years. Swelling (57.9%) and orbital pain (47.4%) were the most common symptoms, followed by erythema (13.2%), vision loss (13.2%), proptosis (7.9%) and diplopia (%7.9). Table summarizes the demographic and clinical characteristics of the patients. Imaging (MRG) was performed in all patients and 63.2% had an orbital biopsy. Orbital imaging revealed extraocular muscles (71.1%), lacrimal glands (50.0%) and optic nerve (42.1%) involvement. Of patients 34.2% had bilateral and 18.4% had retroorbital involvement. The final diagnoses of patients were: IgG4-related disease (34.2%, n =13), idiopathic orbital inflammatory pseudotumor (36.8%, n = 14), granulomatosis with polyangiitis (18.4%, n =7), Sjogren’s syndrome (n=1), relapsing polychondritis (n=1), thyroid-associated orbitopathy (n=1) and fungal granulomatous angiitis (n=1).Conclusion:Inflammatory lesions of the orbit are rare and the diagnosis may be challenging. Differential diagnosis is based on clinical, laboratory, radiologic and histopathologic findings. Although IgG4-related disease is a relatively new diagnostic cause for orbital inflammation, it should be considered more in differential diagnosis.Figure:Disclosure of Interests:Melek Seren Aksun: None declared, Taha Koray Sahin: None declared, Ertugrul Cagri Bolek: None declared, Levent Kiliç: None declared, Elif Günay Bulut: None declared, Kader Karli Oğuz: None declared, Umut Kalyoncu Consultant of: Abbvie, Amgen, Janssen, Lilly, Novartis, UCB, Omer Karadag: None declared

IgG4-related disease in lung: a diagnostic challenge

Pathology ◽  
2020 ◽  
Vol 52 (3) ◽  
pp. 390-392
Author(s):  
Archana Pandita ◽  
Janice Wong
Keyword(s):  
Diagnostic Challenge ◽  
Related Disease ◽  
Igg4 Related Disease

scholarly journals Relapsing Polychondritis or RP-like Symptoms Associated With IgG4-RD. Case Reports and Review of the Literature

2021 ◽  
Author(s):  
Jesica Romina Gallo ◽  
Maria Marcela Schmid ◽  
Josefina Gallino Yanzi ◽  
Sergio Oscar Paira
Keyword(s):  
Autoimmune Disease ◽  
Plasma Cells ◽  
Case Reports ◽  
Relapsing Polychondritis ◽  
Review Of The Literature ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Immune Mediated ◽  
Systemic Disorder

Abstract Relapsing polychondritis (RP) is a rare systemic disorder characterized by recurrent, widespread chondritis of the auricular, nasal, and tracheal cartilages. IgG4-related disease (IgG4-RD) is a systemic immune-mediated disease characterized by the infiltration of IgG4-bearing plasma cells into systemic organs. Although 25% to 35% of patients with RP have a concurrent autoimmune disease. The coexistence of RP and IgG4 is rare considering that, to the best of our knowledge, there are only four previous reports of RP or RP-like symptoms associated with IgG4-RD. (1-4) We herein report two cases which could be RP or RP-like symptoms associated with IgG4-RD.

scholarly journals Corticosteroid Therapy for a Patient with Relapsing Polychondritis Complicated by IgG4-Related Disease

2016 ◽  
Vol 239 (3) ◽  
pp. 223-230 ◽  
Author(s):  
Mari Yamasue ◽  
Shin-ichi Nureki ◽  
Hiroyuki Matsumoto ◽  
Takamasa Kan ◽  
Takehiro Hashimoto ◽  
...  
Keyword(s):  
Corticosteroid Therapy ◽  
Relapsing Polychondritis ◽  
Related Disease ◽  
Igg4 Related Disease
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