scholarly journals IgG4-related disease presenting as recurrent scleritis combined with optic neuropathy

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Su Jin Kim ◽  
Seung Uk Lee ◽  
Min Seung Kang ◽  
Jung Hyo Ahn ◽  
Jonghoon Shin ◽  
...  
Keyword(s):  
Optic Nerve ◽  
Optic Neuropathy ◽  
Rectus Muscle ◽  
Signs And Symptoms ◽  
Organ Damage ◽  
Term Treatment ◽  
Blurred Vision ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Long Term Treatment

Abstract Background We report a case of atypical presentation of IgG4-related disease (IgG4-RD) with recurrent scleritis and optic nerve involvement. Case presentation A 61-year-old male presented with ocular pain and injection in his left eye for 2 months. Ocular examination together with ancillary testing led to the diagnosis of scleritis, which relapsed in spite of several courses of steroid treatment. After cessation of steroid, the patient complained of severe retro-orbital pain and blurred vision. His best corrected vision was count finger, the pupil was mid-dilated and a relative afferent pupillary defect was found. Funduscopic examination demonstrated disc swelling. Magnetic resonance imaging (MRI) showed enhancing soft tissue encasing the left globe, medial rectus muscle and optic nerve. Systemic work-up revealed multiple nodules in right lower lung and a biopsy showed histopathological characteristics of IgG4-RD. Long-term treatment with corticosteroids and a steroid-sparing agent (methotrexate) led to significant improvement in signs and symptoms with no recurrence for 2 years. Conclusions This case highlights the significance of IgG4-RD in the differential diagnosis of recurrent scleritis. IgG4-RD may cause optic neuropathy resulting in visual loss. Early diagnosis and proper treatment can prevent irreversible organ damage and devastating visual morbidity.

scholarly journals IgG4-Related Disease Presenting as Isolated Scleritis

2017 ◽  
Vol 2017 ◽  
pp. 1-4
Author(s):  
Eran Berkowitz ◽  
Ella Arnon ◽  
Alona Yaakobi ◽  
Yuval Cohen ◽  
Beatrice Tiosano
Keyword(s):  
Rare Case ◽  
Signs And Symptoms ◽  
Term Treatment ◽  
Affected Area ◽  
Related Disease ◽  
Eye Pain ◽  
Igg4 Related Disease ◽  
Long Term Treatment ◽  
Steroid Sparing

A rare case of IgG4-related disease (IgG4-RD) manifesting as nodular scleritis is presented in a 20-year-old female. Patient complained of left eye pain and redness for one week. Ocular examination together with ancillary testing led to the diagnosis of nodular scleritis. Since the patient did not show apparent improvement after one week of systemic steroidal treatment, she underwent a biopsy of the affected area revealing histopathological characteristics of IgG4-RD. Long-term treatment with corticosteroids and a steroid-sparing agent (methotrexate) led to significant improvement in signs and symptoms. This case highlights the significance of IgG4-RD in the differential diagnosis of scleritis and raises the question as to whether various organs affected by IgG4-RD may have different underlying pathophysiological mechanisms in which pathogenic T cells play a role.

A Case of Possible IgG4-Related Disease with Bilateral Optic Neuropathy and Bilateral Hippocampal Bleed

2021 ◽  
Vol 69 (2) ◽  
pp. 484
Author(s):  
Anish Mehta ◽  
Sujana Gogineni ◽  
ArjunGaurang Shah ◽  
Selva Kumar ◽  
HH Nagappa ◽  
...  
Keyword(s):  
Optic Neuropathy ◽  
Related Disease ◽  
Igg4 Related Disease

Medical Consequences of Long-term Treatment of Acromegaly

2010 ◽  
Vol 06 ◽  
pp. 68
Author(s):  
Rosario Pivonello ◽  
Renata S Auriemma ◽  
Mariano Galdiero ◽  
Ludovica FS Grasso ◽  
Annamaria Colao ◽  
...  
Keyword(s):  
Signs And Symptoms ◽  
Premature Death ◽  
Term Treatment ◽  
Tumour Mass ◽  
Risk Of Death ◽  
Increased Risk ◽  
Long Term Treatment ◽  
A Minor ◽  
The Impact

This article discusses the impact of long-term treatment of acromegaly on cardiovascular, metabolic, respiratory and articular complications as well as on malignancies. The main goals of treatment of acromegaly include normalisation of biochemical markers of disease activity, improvement in signs and symptoms of the disease, removal or reduction of tumour mass and preservation of pituitary function, together with prevention of complications. Cardiovascular and respiratory complications are the main causes of morbidity and mortality, whereas neoplasms are a minor cause of increased risk of death. Other associated diseases are arthropathy, carpal tunnel syndrome and reproductive disorders. The prolonged elevation of growth hormone (GH) and insulin-like growth factor (IGF)-I levels results in premature death, whereas strong biochemical control improves wellbeing and restores life expectancy to normal.

scholarly journals Ocular Involvement in Sarcoidosis

2017 ◽  
Vol 38 (04) ◽  
pp. 514-522 ◽  
Author(s):  
Aniki Rothova ◽  
Fahriye Groen
Keyword(s):  
Optic Nerve ◽  
Systemic Therapy ◽  
Visual Outcome ◽  
Term Treatment ◽  
Ocular Involvement ◽  
Visual Prognosis ◽  
Long Term Treatment ◽  
Chronic Course ◽  
Optic Nerve Involvement

AbstractOcular involvement in sarcoidosis occurs in ∼40% and the eye is the presenting organ in roughly 20%. The course of ocular disease does not necessarily parallel that of systemic disease. Uveitis is the most common presentation and shows mainly a chronic course; anterior uveitis is associated with better visual prognosis than posterior localization. Painful bilateral anterior granulomatous uveitis most commonly occurs in black patients at younger age, while painless posterior bilateral involvement with peripheral multifocal choroiditis is commonly seen in elderly white females. Patients with posterior uveitis develop often ocular complications and central nervous system involvement. Vitritis, segmental periphlebitis, choroidal granulomas, and peripheral multifocal chorioretinitis are often seen clinical features. Optic nerve involvement is uncommon, but if present, results often in poor visual outcome. Lacrimal gland and conjunctival involvement are also common and present clinically as dry eyes or remain asymptomatic with good visual prognosis. Sarcoidosis-associated uveitis is mostly managed by local treatment with steroid drops or periocular and intraocular steroid injections or with novel intraocular corticosteroid implants. Patients with sight-threatening disease or optic nerve involvement need systemic therapy. Systemic therapy is based on a step-up regimen where corticosteroids are used in the initial phase of the disease and if long-term treatment is required, steroid-sparing immunomodulatory drugs are implemented such as methotrexate or biological agents. Despite the mainly chronic course, need for long-term treatment, and frequent ocular surgeries in the majority of patients, the visual outcome of sarcoidosis-associated uveitis is fairly good if therapy has started on time.

scholarly journals IgG4-Related Disease Manifesting as Hypocomplementemic Tubulointerstitial Nephritis: A Rare Case Report and Literature Review

2020 ◽  
Vol 8 ◽  
pp. 232470962095221
Author(s):  
Pradnya Brijmohan Bhattad ◽  
David L. Joseph ◽  
Eric Peterson
Keyword(s):  
Kidney Disease ◽  
Vision Loss ◽  
Tubulointerstitial Nephritis ◽  
Organ Damage ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Multiple Organs ◽  
Igg4 Related Disease ◽  
Rare Case Report ◽  
Progression Of Kidney Disease

Immunoglobulin G4–related disease (IgG4-RD) is a chronic fibrosing inflammatory systemic disorder that has been recognized relatively recently in the medical literature. Little is known about the exact disease pathogenesis and epidemiology. IgG4-RD may be asymptomatic or may have minimal symptoms or involve multiple organs with overt symptoms. The different phenotypes of IgG4-RD can lead to delayed or incorrect diagnosis. We report the case of a 66-year-old male with coal worker’s pneumoconiosis who presented with progressive kidney disease and was diagnosed with tubulointerstitial nephritis due to IgG4-RD. The patient was noted to have progressive kidney disease, skin involvement, worsening interstitial lung disease, complete vision loss in the left eye, and retroperitoneal fibrosis. Serologic workup revealed elevated inflammatory markers, IgG4 and IgG1 levels, and hypocomplementemia. A tissue biopsy helped us establish a definitive diagnosis of IgG4-RD and initiate treatment with glucocorticoids to prevent further progression of kidney disease and other end-organ damage.

scholarly journals Optic neuropathy from connected intra- and extraorbital lesions in IgG4-related disease

2019 ◽  
Vol 59 (11) ◽  
pp. 746-751 ◽  
Author(s):  
Tsuneaki Yoshinaga ◽  
Toru Kurokawa ◽  
Takeshi Uehara ◽  
Junpei Nitta ◽  
Tetsuyoshi Horiuchi ◽  
...  
Keyword(s):  
Optic Neuropathy ◽  
Related Disease ◽  
Igg4 Related Disease
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