1. Case report Conclusion Pictures Discussion Reference
IgG4 related disease with orbital mass - A case report
Chao-Wei Chuang, MD ; Kuan-Chih Hung, MD
Department of Ophthalmology, Chia-Yi Christian Hospital
莊超偉 洪冠智
嘉義基督教醫院眼科
Case report
Fig. 6 showed the IgG4-positive plasma cells infiltration and it is not focal aggregations. Due to previously finding, the IgG4-realted disease is impressed. Most clinical manifestations of IgG4-related disease
respond to glucocorticoids. These agents are the first-line, standard-of-care approach for most patients. The other medication included azathioprine、mycophenolate mofetil and methotrexate.
This 34 years old male denied any systemic disease or autoimmune disease before. This time he complained of the bilateral upper eyelid swelling for many months. In our clinic, there were no parotid gland or submandibular gland swelling found. There were also no extraocualr movement limitation , lid retraction or lagophthalmos found. The intraocular pressure and corrected visual acuity were not effected. Physical examination showed the palpebral mass at superior-temporal area (Fig. 1).
The orbital magnetic resonance imaging (MRI) reported the enlargement of the bilateral lacrimal glands with homogeneous enhancing soft tissue masses (Fig. 2). The oribtal solid tumor masses at bilateral lacrimal glands is considered, so we arranged excision and biopsy for him. The pathology report revealed the lacrimal glandular tissue (Fig. 3) with dense lympho-plasma-cytic infiltrate, mild-to-moderate eosinophilic infiltrate (Fig. 4), storiform fibrosis (Fig. 5) and advanced lymphocytic infiltrate, organizied in germinal centers. Further immunology stain reported positive findings of cluster of differentiation (CD) 3, CD20, Kappa and Lambda light chain and immunoglobulin G4 (IgG4) (Fig. 6). The IgG4 related disease was impressed. We prescribed prednisolone and rituximab for him, and serum IgG4 leverl decreased gradually.
Conclusion
IgG4-related disease is recognized in recently 10 years. The disease may response well to steroid treatment.
Pictures
Figure 1 palpebral mass at superior-temporal area
Figure 2 ocular MRI revealed
soft tissue masses .
Discussion
Figure 3 lacrimal glandular tissue
Figure 4 eosinophilic infiltrate
IgG4-related disease is a protean condition that mimics many malignant, infectious, and inflammatory disorders. This multi-organ immune-mediated condition links many disorders previously regarded as isolated, single-organ diseases without any known underlying systemic condition. It was recognised as a unified entity only 10 years ago. Histopathology is the key to diagnosis. The three central pathology features of IgG4-related disease are lymphoplasmacytic infiltration, storiform fibrosis, and obliterative phlebitis. The extent of fibrosis is an important
determinant of responsiveness to immunosuppressive therapies.
In our case, there were only lacrimal galnds involved at present. The orbital MRI revealed the mass, but tissue biopsy is the gold standard for diagnosis in most settings. In IgG4-related disease, The lymphocytes and plasma cells are polyclonal. Eosinophils are also commonly present and extreme examples can resemble eosinophilic organopathy, but neutrophilic infiltration is rare in IgG4-related disease. Fig. 3 illustrated the lympho-plasma-cytic infiltrate with germinal center. The positive immunology stain finding of CD3 and CD20 indicated the B-cell infiltration. The positive result of Kappa and Lambda light chain stain revealed the polyclonal lymphocytes. Fig.3 and Fig. 5 showed the storiform fibrosis. There were no obliterative phlebitis found. It may due to that the sample size is too small to find out the obliterative phlebitis.
Figure 5 storiform fibrosis
Figure 6 IgG4 stain
Reference
Kamisawa, Terumi, et al. "IgG4-related disease." The Lancet  (2015):