IgG4-related disease: a contemporary review

Abstract

Immunoglobulin G4-related disease (IgG4-RD), is an immune-mediated fibroinflammatory condition, which may involve multiple organs and mostly presents with high serum IgG4 levels and specific histopathological characteristics. As IgG4-RD is a relatively new entity the etiology, prevalence and epidemiologic knowledge is quite limited. Although involvement of almost all anatomical regions has been reported, the most commonly affected regions are pancreas, lacrimal glands, salivary glands, retroperitoneum, orbita, lymph nodes, kidney and lungs. Diagnosis is made with combined evaluation of clinical, radiological and histopathological findings. Typical histopathological features include storiform fibrosis, dense lymphoplasmacytic infiltrates and obliterative phlebitis. Its course is typically marked by remission and relapsing attacks and it may lead to fibrosis, destructive lesions in tissues and organ failure unless promptly treated. In the treatment of IgG4-RD, many approaches including surgical resection of tissues, systemic glucocorticoids, steroid-sparing immunosuppressive drugs, and biological agents are employed. Although association is not clear, malignancies are frequently reported in IgG4-RD patients. Therefore, it is prudent to monitor patients for the symptoms of malignant diseases.

Sinonasal immunoglobin G4—related disease: case report and review

“…Prednisone monotherapy at a dose of 0.6 mg/kg/ day and tapered over the period of 3-6 months is currently considered the first-line therapy. 26 In a recent study, the recurrence rate for IgG4-RD was reported to be 46% after initial treatment with steroids. However, this review of sinonasal disease demonstrated that, of the 14 patients who initially underwent steroid monotherapy, only 1 had recurrent disease (7%).…”

Sinonasal IgG4-RD: Case Report and Systematic Review

“…Prednisone monotherapy at a dose of 0.6 mg/kg/day and tapered over the period of 3-6 months is currently considered the first-line therapy. 26 In a recent study, the recurrence rate for IgG4-RD was reported to be 46% after initial treatment with steroids. 26 However, this review of sinonasal disease demonstrated that, of the 14 patients who underwent steroid monotherapy initially, only 1 had recurrent disease (7%).…”

“…26 In a recent study, the recurrence rate for IgG4-RD was reported to be 46% after initial treatment with steroids. 26 However, this review of sinonasal disease demonstrated that, of the 14 patients who underwent steroid monotherapy initially, only 1 had recurrent disease (7%). In that case, the addition of rituximab led to clinical improvement.…”

Pseudotumoral Lesion as a Manifestation of Autoimmune Pancreatitis

“…Patients can present with subacute development of a mass in the affected organ, even though 60% to 90% of these patients have multiple organ involvement [1]. The prevalence of IgG4-RD is unknown since it is a relatively new entity and still largely underrecognized in clinical practice [2].…”

“…The IgG4-RD associated pancreatitis is known as type 1 AIP. IgG4-related endocrinopathies are rare; however, in type 1 AIP, endocrine and exocrine dysfunction develop commonly, manifesting with hyperglycemia and steatorrhea [2]. AIP-related hyperglycemia may be completely controlled after glucocorticoid administration [3].…”