Background/Purpose: Pathology associated with IgG4-related disease (IgG4-RD) has been described in virtually every tissue and organ of the body. The heterogeneous clinical manifestations of IgG4-RD are a consequence of the variation in clinical presentation for any given IgG4-RD patient. The clinical presentation in early IgG4-RD is rarely definitive especially in the absence of more obvious manifestations such as pancreatitis. For example, more apparent clinical presentations such as swollen salivary glands and lymph nodes, swollen eyelids, and intraocular discomfort may not be readily diagnosed as IgG4-RD. When combined with prevalent tumefactive modules in the lung and/ spleen, all of these clinical manifestations are diagnostic for IgG4-RD. Swelling or nodules of multi-sites combined with histopathological features of tumefactive lesions and laboratory examinations to exclude neoplasm and infection may improve better and more rapid diagnosis of IgG4-RD. Given the propensity of IgG4-RD to progressively affect more tissues and organs, the latter concern is critical.

Methods: We retrospectively examined the clinical and laboratory records for 59 patients with definite IgG4-RD collected from all departments of West China Hospital between January 2012 and December 2017.

Results: A total number of 59 patients were enrolled, including male 41 (70%), female 18 (31%). The maximum and minimum onset age were 85 and 15 respectively. The majority of patients had multiple sites and/or organs involved: 90% of patients were suffering from 3 and more sites or organs affected; 8.5% of patients, 2 sites or organs involved; and only 1.7% with single site or organ involvement. The mostly commonly recorded pathologies involved sites were lung, manifested as small nodules (71%) and lymph nodes (54%). Other involved sites included: salivary glands (42%) which included submandibular (29%), parotid (12%), and labial (1.7%) glands; kidney (36%); pancreas (32%); periorbital tissues (31%) including intraorbital lesions (14%) and swollen eyelids (17%); liver (27%); biliary tree (19%); spleen (15%); nasal sinus (15%); lacrimal glands (15%); and thyroid glands (14%). Other involved tissues included gastrointestinal tract, skin, glottis/pharynx, peritoneum, pericardium, pleura, and large arteries (3.4-8.5%). Rarely involved sites were gingiva, spine, pituitary glands, and mediastinum (1.69%). Elevated serum IgG4 was found in 44 (75%) patients, and antinuclear antibody in 17 (29%) of patients.

Conclusion: Almost any tissue or organ can be affected in IgG4-RD. The most commonly involved organs identified in this retrospective study were lung, lymph node, salivary glands, kidney, and pancreas, respectively. Clinical manifestations may be more obvious with pancreatitis and enlarged lymph nodes; however, small nodules in lung, liver and spleen may be overlooked due to the absence of accompanying symptoms. Our goal is to increase awareness among physicians in general that tumefactive lesions in any site or organ, especially if they occur in multiple tissues or organs, in asymptomatic early stage disease may be indicators for diagnosis of IgG4-RD.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/how-to-better-diagnose-igg4-related-disease-a-single-center-based-experience/