scholarly journals IgG4-associated disease in differential diagnosis of inflammatory orbitopathy

2019 ◽  
Vol 65 (5) ◽  
pp. 367-372
Author(s):  
Yaroslav O. Grusha ◽  
Dilyara S. Ismailova ◽  
Natalya Yu. Sviridenko ◽  
Pavel I. Novikov ◽  
Alla M. Kovrigina

IgG4-associated disease (IgG4-RD) is a systemic inflammatory disease characterized by tumorlike sclerosing masses in different organs. Differential diagnosis in orbital IgG4-RD includes majority of conditions, such as thyroid eye disease (TED), sarcoidosis, granulomatosis with polyangiitis, idiopatic orbital inflammation, limphoproliferative diseases and others. A case of IgG4-RD with different organs involvement and complicated differential diagnosis is presented. This case demonstrates very uncommon manifestation of IgG4-RD, when orbital involvement was very similar with TED. Systemic process was not recognized during a long period of time and diagnosis of IgG4-RD was established only after biopsy of abnormally increased lacrimal gland. Differential diagnosis included other systemic diseases, first of all sarcoidosis, GPA, and lymphoma. Biopsy results were consistent with the gold standard of diagnosis, e. g. more than 40% of plasma cells were IgG4 positive. This case demonstrates the necessity of orbital biopsy before starting immunosuppression to avoid inappropriate treatment strategy.

2012 ◽  
Vol 2012 ◽  
pp. 1-6 ◽  
Author(s):  
Sing Yun Chang ◽  
Karina Keogh ◽  
Jean E. Lewis ◽  
Jay H. Ryu ◽  
Eunhee S. Yi

Granulomatosis with polyangiitis (Wegener’s) (GPA) may mimic IgG4-related disease (IgG4-RD) on histologic examination of some biopsies, especially those from head and neck sites. IgG4 immunostain is often performed in this context for differential diagnosis with IgG4-RD. However, the prevalence of IgG4+ cells in GPA has not been explored. We examined the IgG4+ cells in 26 cases confirmed as GPA by a thorough clinical and pathologic assessment. Twenty-six biopsies consisted of 14 sinonasal/oral cavity/nasopharynx, 7 orbit/periorbital, 3 lung/pleura, 1 iliac fossa/kidney, and 1 dura specimens. Eight of 26 (31%) biopsies revealed increased IgG4+ cells (>30/HPF and >40% in IgG4+/IgG+ ratio). The IgG4+ cells and IgG4+/IgG+ ratio ranged 37–137/hpf and 44–83%, respectively. Eight biopsies with increased IgG4+ cells were from sinonasal(n=4)or orbital/periorbital(n=4)sites. In conclusion, increased IgG4+ cells are not uncommonly seen in sinonasal or orbital/periorbital biopsies of GPA, which could pose as a diagnostic pitfall.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1813.1-1813
Author(s):  
B. Lamoreaux ◽  
M. Francis-Sedlak ◽  
R. Holt ◽  
J. Rosenbaum

Background:Autoimmune inflammatory conditions of the eye may be associated with rheumatic diseases such as rheumatoid arthritis, systemic lupus erythematosus, and granulomatosis with polyangiitis. This is also observed with thyroid eye disease (TED). Loss of immune tolerance to the thyroid stimulating hormone receptor has thyroidal consequences and nearly 40% of patients with Graves’ disease also have clinically evident Graves’ orbitopathy or TED.1TED results from tissue inflammation that causes retro orbital fat expansion2and extraocular muscle enlargement2and stiffening.3Because the orbital cavity is bony and of limited volume, proptosis and, in severe cases, optic nerve compression, can result. In many patients, muscle changes also cause ocular motility issues and double-vision. Because TED can have a similar presentation to other inflammatory orbital diseases (e.g., granulomatosis with polyangiitis) and Graves’ disease patients frequently have other autoimmune conditions (10% of Graves’s patients also have rheumatoid arthritis),4rheumatologists are likely to care for, or even diagnose, patients with TED.Objectives:This analysis sought to understand rheumatologists’ knowledge, and degree of participation in the treatment, of TED including referral patterns from ophthalmologists and endocrinologists for infusion therapies.Methods:Rheumatologists practicing in the United States attended an educational session and agreed to complete a 12-item survey regarding TED awareness, referral patterns, and management.Results:Of the 47 rheumatologists surveyed, 45 (96%) were familiar with TED. Ten (21%) physicians reported managing patients with TED, but the majority of physicians (62%) reported that they co-managed other autoimmune diseases in patients who also had TED. Additionally, 98% and 64% of polled rheumatologists had received referrals from ophthalmologists and endocrinologists, respectively, for autoimmune disease management or infusion therapy. Ophthalmology referrals for intravenous (IV) medication administration were most frequently for biologics (82%), but some referrals were also made for corticosteroids (2%) or other medication (13%) infusions. Only 23% of rheumatologists had administered a biologic specifically for TED (rituximab: 17%, tocilizumab: 2%, other: 4%), but 89% expressed an interest in administering a TED-specific monoclonal antibody therapy, awaiting FDA approval.Conclusion:Nearly all surveyed rheumatologists were aware of the signs and symptoms of TED, although most did not actively manage or administer medication for TED. Given the high level of interest in infusing novel, TED-specific biologics, rheumatologists may become an integral part of TED patient management with the approval of a new biologic, teprotumumab, for thyroid eye disease.References:[1]Bartley GB, et al.Am J Ophthalmol1996;121:284-90.[2]Forbes G, et al.AJNR Am J Neuroradiol1986;7:651-656.[3]Simonsz HJ, et al.Strabismus1994;2:197-218.[4]Cardenas Roldan J, et al.Arthritis2012 2012;864907.Disclosure of Interests:Brian LaMoreaux Shareholder of: Horizon Therapeutics, Employee of: Horizon Therapeutics, Megan Francis-Sedlak Shareholder of: Horizon Therapeutics, Employee of: Horizon Therapeutics, Robert Holt Shareholder of: Horizon Therapeutics, Employee of: Horizon Therapeutics, James Rosenbaum Consultant of: AbbVie, Corvus, Eyevensys, Gilead, Novartis, Janssen, Roche, UCB Pharma; royalties from UpToDate


2011 ◽  
Vol 135 (12) ◽  
pp. 1597-1600 ◽  
Author(s):  
John J Cangelosi ◽  
Victor G Prieto ◽  
Doina Ivan

Rosai-Dorfman disease (RDD) is characterized histologically by a dense histiocytic infiltrate with emperipolesis and associated lymphocytes, plasma cells, and neutrophils. Eosinophils are not commonly associated. We report a patient with initial thymus and pituitary gland involvement by RDD, who later developed papules on the groin and axilla. Skin biopsies showed admixed histiocytic infiltrates (lymphocytes, neutrophils, and plasma cells) without emperipolesis. A prominent eosinophilic infiltrate was also observed, a feature not, to our knowledge, previously reported. Immunohistochemistry revealed positivity for CD68 (most cells) and S100 protein (scattered cells) and was negative for anti-CD1a. The diagnosis of RDD was established in the clinical context after comparison with the thymic and pituitary lesions (similar histologic features, albeit with fewer eosinophils, and immunohistochemical profiles). We present the first case, to our knowledge, of multicentric RDD with cutaneous involvement and associated prominent eosinophilic infiltrate. Thus, RDD should be included in the differential diagnosis of mononuclear infiltrates containing eosinophils.


1987 ◽  
Vol 1 (1) ◽  
pp. 33-44 ◽  
Author(s):  
Guy A. Settipane

Many systemic diseases are associated with nasal symptoms, Rhinitis associated with asthma is probably the most common with leprosy and fungal infections being the rarest. A careful history and nasal examination in a patient with rhinitis may lead to the discovery of more significant systemic diseases. Proper treatment of systemic disease will often cure or improve the associated rhinitis. Similarly, appropriate treatment of the rhinitis/sinusitis may reduce systemic complaints such as asthma. At times, identification of the cause of rhinitis as in CSF rhinorrhea, Wegeners’ syndrome, etc., alerts one to a life-threatening entity. Thus, it is apparent that the nose is an excellent mirror of some systemic diseases and identifying and understanding the differential diagnosis of nasal symptoms may be a tremendous help in diagnosing the disease and treating the whole patient.


2021 ◽  
Vol 15 (8) ◽  
pp. 2057-2059
Author(s):  
Maham Ashraf ◽  
Aysha Anjum ◽  
Eisha Tahir ◽  
Amber Goraya ◽  
Rabia Aqeel

Background & Objective: Renal tumors are a common finding in diagnostic imaging; these lesions usually are solid or cystic, benign or malignant, and the correct diagnosis may be difficult. The current study aims at to determine the diagnostic accuracy of magnetic resonance imaging for the diagnosis of Wilms tumor taking histopathology as gold standard. Methodology: This cross-sectional study was carried at the Department of Radiology, The Children’s Hospital & Institute of Child Health Lahore over 6 months from March 2019 to September 2019. The study involved 125 children of both genders aged between 2 years to 14 years presenting with a neoplastic mass in the retroperitoneum on ultrasound abdomen during initial workup. These children were assessed on MRI for the diagnosis of Wilms tumor. Later the diagnosis was confirmed on histopathology which was taken as gold standard and the diagnosis of MRI was judged accordingly as true/false and positive/negative. A written informed consent was obtained from every patient. Results: The mean age of the children was 5.8±3.9 years having a male predominance with male to female ratio of 1.8:1. Wilms tumor was suspected in 13 (10.4%) children on MRI. The diagnosis of Wilms tumor was confirmed in 13 (10.4%) children on histopathology. MRI was found to be 76.9% sensitive, 97.3% specific and 95.2% accurate with positive and negative predictive values of 76.9% and 97.3% respectively. Conclusion: In the present study, MRI was found to be 95.2% accurate in the differential diagnosis of Wilms tumor in children presenting with retroperitoneal mass which along with its non-invasive and radiation free nature advocates the preferred use of MRI in the diagnostic evaluation of such children in future oncologic practice. Keywords: Retroperitoneal Tumor, Wilms Tumor, MRI, Diagnostic Accuracy


2016 ◽  
Vol 32 (1) ◽  
pp. 39-42
Author(s):  
Md Atikur Rahman ◽  
Aklaque Hossain Khan ◽  
Kanak Kanti Barua

Primary craniocerebral plasmacytomas are uncommon and represent only 0.7 % of all plasmacytomas. In this case solitary plasmacytoma in the midline frontal head region of the skull and discuss the clinical features and prognosis of this tumor. Plasmacytoma can present as multiple myeloma, solitary plasmacytoma of the bone or extramedullary plasmacytoma. Solitary plasmacytoma is a rare entity that composes of malignant plasma cells and involves the bone to form only one or two lesions without evidence of disease dissemination. It accounts for only 4% of malignant plasma cell tumors. 50 years old male was suffering from plasmacytoma in the frontal head region in our case which is pulsatile. On images showed multiple differential diagnosis but after operation histological examination revealed plasmacytoma. Bangladesh Journal of Neuroscience 2016; Vol. 32 (1): 39-42


Author(s):  
Laparra-Galindo Julia Patricia ◽  
Mendez-Valenzuela Carlos Roberto

The goal of this article is to show the most frequent types of differential diagnoses of systemic diseases in canines with ocular signs and lesions. For this purpose, a retrospective study was conducted on 510 medical records from patients of the Companion Animal Veterinary Hospital (HVAC) of the University of San Carlos (USAC) of Guatemala, from August to November 2017. The inclusion criteria were: canine species, having a differential diagnosis established list of possible systemic diseases and an eye examination by specialist. Through a descriptive analysis of the data, it was established that, of the 510 canines examined, 212 (42%) presented signs and ocular alterations, of these there were 550 differential diagnosis, which were categorized using the acronym DAMNIT V; from which it was determined that 40% are infectious / inflammatory, neoplasic (19%) followed by metabolic processes (13%), allergic / autoimmune (10%), degenerative (7%), idiopathic (5%), traumatic / toxic 4% and finally vascular (2%).


Posterior scleritis is a painful inflammatory disease that affects the sclera behind the ora serrata. Although it can be seen at any age, it is mostly seen over the age of 40 and mostly affects women. It is usually unilateral. The most common symptoms are periocular pain and loss of vision. It is often idiopathic but it may be associated with systemic diseases such as rheumatologic and autoimmune illnesses. Because of its variable clinical features, the differential diagnosis should be done with orbital or intraocular inflammation and ocular tumors. B-mode ultrasonography (USG), computed tomography, and magnetic resonance imaging are helpful in diagnosis. Posterior scleritis should be kept in mind in all inflammatory and painful ocular disorders without a significant etiology. With the correct diagnosis, appropriate and aggressive treatment, it is possible to recover and protect vision.


Author(s):  
A. Ghare ◽  
F. Haji ◽  
K. MacDougall

Plasmacytomas are solitary tumours characterized by neoplastic proliferation of plasma cells and can be found isolated or in associated with multiple myeloma. Plasmacytomas uncommonly occur intracranially, and dural plasmacytomas without involvement of the calvarium are exceedingly rare. Reported cases indicate durally-based plasmacytomas mimick the appearance of meningioma, lymphoma or sarcoma of the dura. The authors report a case of a 77-year-old male with known multiple myeloma who presented with a 3-week history of confusion, speech impediment, and right sided weakness. A non-contrast CT scan revealed a dense extra-axial mass in the left frontal lobe with initial concerns of an extra-axial hemorrhage. A subsequent MRI demonstrated a contrast enhancing mass with a broad-based dural tail and no underlying calvarial lesion. Differential diagnosis included meningioma or intracranial plasmacytoma. The patient underwent surgical resection and was found to have intratumoural hemorrhage, with pathology confirming plasmacytoma. In the published literature, there are only 20 prior reports of dural plasmacytomas (with and without primary calvarial infiltration), of which only five previous cases reported associated intratumoural hemorrhage. Our case, along with this literature, suggests that new onset of focal neurologic deficits in patients with a history of multiple myeloma merits careful investigation, and that intracranial plasmacytoma should be considered on the differential diagnosis even when imaging reveals masses consistent with hemorrhage or meningioma.


2020 ◽  
Vol 99 (10) ◽  
pp. 658-663
Author(s):  
Federica Perardi ◽  
Giuseppe Abbate ◽  
Leonardo R. Iannuzzelli ◽  
Rossella Contini ◽  
Manuela De Munari ◽  
...  

Angioleiomyoma is a benign smooth muscle and vessel tumor; laryngeal localization is extremely rare with only 24 cases described in the literature; moreover, it should be considered in the differential diagnosis of laryngeal mass. Endoscopic complete surgical excision with dissection along capsule is now considered the gold-standard treatment for small and well-circumscribed laryngeal angioleiomyoma. We present a case of laryngeal angioleiomyoma successfully treated with carbon dioxide laser technology which resulted in a bleeding reduction and adequate hemostasis with less tissue damage and good functional outcome.


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