The variety of clinical presentations in IgG4-related disease in Rheumatology

Abstract

IgG4-related disease (IgG4-RD) belongs to the group of rare diseases in which the identification of the characteristic histology and immunohistochemistry provides with the gold standard in the diagnosis. The variable organ dysfunction reflects the clinical presentation. The examples of different IgG4-RD presentations in the Rheumatology Unit were discussed in this article. The spectrum of IgG4-RD is wide-ranging and manifested in one or more organs synchronously or metachronously. In the presented article, we described five different cases of IgG4-RD. Four cases were reaffirmed in the histopathological assessment. The clinical and laboratory findings were analyzed and the assigned therapy was discussed. According to our experience, the diagnosis of IgG4-RD requires the careful clinicopathological correlation. The diagnosis relies on the coexistence of various clinical, laboratory, radiological, and histopathological findings, although none of them is pathognomonic itself. The time needed for the diagnosis and variety of clinical forms of IgG4-RD shows that there is need of the cooperation among many specialists for the better and earlier recognition of the disease.

Keywords: Differential diagnosis; IgG4; Pathology; Treatment.

Fig. 1

The lacrimal gland with the dense lymphoplasmacytic infiltration and advanced fibrosis, which are highly histologically suggestive for IgG4-related disease (a 200×, H&E staining). The inflammatory infiltration in the presented above lacrimal gland is predominantly consisted of the IgG4-positive plasma cells (b 200×, hematoxylin). Immunostaining for the IgG4 in the salivary gland in one high-power field (hpf), which is an accepted method for counting of the IgG4-positive plasma cells based on Deshpande et al. [6] (c 400×, hematoxylin). The increased number of IgG4-positive plasma cells in the salivary gland (d): 112 IgG4-positive plasma cells based on the detailed counting with Cell^D Program (Olympus, Poland)

Fig. 2

36-year-old female patient. The characteristic changes of IgG4-RD (a and b). It is the typical Mikulicz disease (MD) with the lacrimal glands enlargement and salivary glands enlargement which deformed normal features. The improvement after 3 weeks of the treatment with glucocorticoids (c and d)

Fig. 3

The lacrimal gland enlargement in the patient with the simultaneously diagnosed ileus pseudotumor