Diagnostic and treatment workup for IgG4-related disease

Abraham, Mary; Khosroshahi, Arezou

doi:10.1080/1744666x.2017.1354698

Cited by 42 publications

(47 citation statements)

References 67 publications

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“…Advances in understanding the disease pathogenesis have given some promising ideas which may lead to new therapeutic options, such as bortezomib, abatacept and dupilumab[ 34 - 38 ]. Infliximab was recently proposed for the treatment of IgG4-related orbital disease refractory to GC[ 35 , 39 ].…”

Section: Discussionmentioning

confidence: 99%

Multi-organ IgG4-related disease continues to mislead clinicians: A case report and literature review

Strainiene¹,

Sarlauskas²,

Savlan³

et al. 2020

WJCC

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BACKGROUND Immunoglobulin G4-related disease (IgG4-RD) is a multisystemic mass forming immune-mediated disease that affects almost every organ and is a diagnostic challenge for every clinician. There is a lack of adequate epidemiological data worldwide, and evidence-based treatment recommendations are not yet established. We report the first case of IgG4-RD from Lithuania and the Baltic Sea region presented with thyroiditis, orbital myositis, orbitopathy, uveitis, scleritis, sialadenitis, autoimmune pancreatitis and prostatitis. CASE SUMMARY A 54-year-old Caucasian male was admitted to our tertiary Centre complaining of severe weight loss, diarrhoea, abdominal pain, salivary gland swelling, sicca symptoms and diplopia. On examination, bilateral palpable masses in the projection of major salivary glands, severe protrusion of the left eyeball and cachexia were noted. The patient was previously diagnosed with autoimmune thyroiditis and endocrine ophthalmopathy. The magnetic resonance imaging (MRI) of the head revealed enlarged extraocular muscles indicating orbital myositis. The biopsy from the salivary gland mass indicated sialadenitis. Abdominal MRI showed signs of autoimmune pancreatitis, and a serological test revealed the elevated serum IgG4 concentration. The patient was then diagnosed with IgG4-RD and successfully treated with prednisolone. There was a significant clinical, serological and radiological improvement after one month of treatment and no signs of relapse within twenty months. However, it took almost 18 years and the efforts of eight different medical specialists to establish the correct diagnosis. CONCLUSION A comprehensive approach to the patient is essential to improving the recognition of rare immune system conditions, such as IgG4-RD.

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Section: Discussionmentioning

confidence: 99%

Multi-organ IgG4-related disease continues to mislead clinicians: A case report and literature review

Strainiene¹,

Sarlauskas²,

Savlan³

et al. 2020

WJCC

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“…a.) in Betracht kommt, alternativ eine Erhaltungsdosis von 5-7,5 mg Prednison über mehrere Monate [13]. Vergleichende Studien zur Wirksamkeit verschiedener Schemata gibt es bisher nicht.…”

Section: Merkeunclassified

Orbitale Entzündung: aktuelle Trends

Pitz¹

2020

Augenheilkunde up2date

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ZusammenfassungIn das Feld der idiopathischen orbitalen Entzündung ist „Bewegung“ gekommen: Mit der Erstbeschreibung der IgG4-assoziierten Erkrankung vor etwa 10 Jahren ist die Gruppe der idiopathischen Läsionen um etwa die Hälfte geschrumpft. Die Differenzialdiagnose ist deutlich anspruchsvoller geworden, weil die klinische und bildgebende Überlappung dieser Krankheitsbilder erheblich ist. Der vorliegende Artikel gibt einen Überblick über dieses im Umbruch befindliche Feld.

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“…Taking the above into consideration, diagnostic and therapeutic procedures were developed in 2015 in patients with suspected IgG4-RD, which is presented in Figure 1 [22,54].…”

Section: Criteria Of Igg4-rd Diagnosismentioning

confidence: 99%

“…Mikulicz's disease is an enlargement (usually symmetrical) of lacrimal glands, parotid and submandibular glands, and sometimes sublingual glands. In the past, Mikulicz's disease was Diagnostic scheme in IgG4-RD [22,37,54].…”

Section: Igg4-rd Of the Head And Neckmentioning

confidence: 99%

IgG4-Related Disease and the Spectrum of Mimics in Rheumatology

Sebastian¹,

Donizy²,

Wiland³

2019

Chronic Autoimmune Epithelitis - Sjogren's Syndrome and Other Autoimmune Diseases of the Exocrine Glands

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Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is an immune-mediated condition that can affect almost any organ. It is a chronic, systemic, inflammatory condition of unknown etiology. Pseudotumor formation is the most common and characteristic clinical symptom. The variable organ dysfunction reflects the clinical presentation. Because there are not specific antibodies for this disease, histopathological assessment provide the pivotal role in the diagnosis. IgG4-RD is characterized by a lymphoplasmacytic infiltrate composed of IgG4+ plasma cells, storiform fibrosis, obliterative phlebitis and mild to moderate eosinophilia. In this chapter we present the newest knowledge of the IgG4-RD pathogenesis and then concentrate on clinical symptoms which can mimic many other conditions in rheumatology, e.g., this common as Sjӧgren syndrome or rare as vasculitis or idiopathic retroperitoneal fibrosis.

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Diagnostic and treatment workup for IgG4-related disease

Cited by 42 publications

References 67 publications

Multi-organ IgG4-related disease continues to mislead clinicians: A case report and literature review

Multi-organ IgG4-related disease continues to mislead clinicians: A case report and literature review

Orbitale Entzündung: aktuelle Trends

IgG4-Related Disease and the Spectrum of Mimics in Rheumatology

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