Sinonasal IgG4‐related sclerosing disease: A rare entity and challenging diagnosis

Abstract

Objectives To describe the rare presentation, imaging and histological findings, and treatments in patients with IgG4‐related disease (IgG4‐RD) and diagnostic pitfalls and difficulties. Methods Cases of sinonasal IgG4‐RD were retrieved, and clinicopathological features were reviewed. Results Seven cases of sinonasal IgG4‐RD were identified over an 11‐year period, including four males and three females, with an age range of 19–66 years (median 58 years). Patients presented with symptoms related to the mass effect of the lesions or the destructive nature of the disease including fullness, swelling, obstruction, and pain. Serum IgG and IgG4 levels, IgG/IgG4 ratios, storiform fibrosis, obliterative phlebitis, and plasma cell infiltration were seen in varying proportions. Bony erosion and tissue inflammation were present in some cases. Conclusion Sinonasal IgG4‐RD is exceedingly rare among other IgG4‐RD and varied in its clinical presentation thus posing as a clinically difficult disease to diagnosis. Proper clinical, pathological, and immunohistopathological analysis is required for accurate diagnosis. Such disease should be considered in all cases of similar presentation to those in this study. Level of Evidence: 4.

Sinonasal schwannoma masquerading as an IgG4-related nasal mass

“…The basic pathology of the condition is related to tissue fibrosis caused by infiltration by IgG4 plasma cells. The head-and-neck region is the most common site after pancreas; within this region, orbits are most commonly involved 5 6…”

“…Surgical management is primarily targeted at getting a tissue sample for biopsy as inflammation may be diffuse and surgical planes may be ill-defined. Wider resection is advised only to reduce related complications (proptosis, motility restriction, mass effect) and complete resection is usually not targeted 5 6 8…”

“…Systemic investigations included complete blood count and a metabolic work-up for diabetes control status. Serum IgG4 level was 4.1 g/L (raised; cut-off >1.35 g/L) 5. Autoimmune work-up for inflammatory diseases was unremarkable (normal serum levels for ANA, c-ANCA, p-ANCA, C-Reactive protein, rheumatoid arthritis factor, serum ACE (ACE) level); ESR (erythrocyte sedimentation rate) was raised to 35 mm/first hour.…”

“…Serology for raised IgG4 levels is inconsistent as 20–40% patients may have normal serum IgG4 levels. Concentration >135 mg/dL may be indicative of disease but raised levels may also be seen in atopic dermatitis, pemphigus, Sjogren syndrome, asthma, hypereosinophilic syndrome, Behcet disease, and multicentric Castleman disease 5–8…”

Sinonasal immunoglobulin G4-related disease: a case report of an atypical and rare entity

“…Various organs, including the lacrimal, salivary, and thyroid glands, pancreas, biliary tract, and retroperitoneum, can be affected [3]. Conversely, the occurrence of IgG4-RD as a solitary sinonasal lesion is exceedingly uncommon in existing literature [4][5][6]. Clinical manifestations of sinonasal IgG4-RD often include facial discomfort, nosebleeds, and persistent rhinosinusitis [7].…”