Systemic corticosteroid therapy yields good clinical response although immunosuppressive or B-cell depleting drugs are often needed.

Patient with refractory anti-CD20 therapy may be assessed for a) other modalities for B cell depletion or b) low grade B cell lymphoma or other potential etiologies

Lancet. 2014 Dec 3.  [Epub ahead of print]

IgG4-related disease.

Kamisawa T1, Zen Y2, Pillai S3, Stone JH4.

Abstract

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. IgG4-related disease generally responds to glucocorticoids in its inflammatory stage, but recurrent or refractory cases are common. Important mechanistic insights have been derived from studies of patients treated by B-cell depletion. Greater awareness of this disease is needed to ensure earlier diagnoses, which can prevent severe organ damage, disabling tissue fibrosis, and even death. Identification of specific antigens and T-cell clones that drive the disease will be the first steps to elucidate the pathogenesis of IgG4-related disease.

J Neuroophthalmol. 2014 Dec;34(4):400-7.

IgG4-related disease: a neuro-ophthalmological perspective.

Kashii S1.

Abstract

Immunoglobulin G4-related disease (IgG4-RD) is a multifocal inflammatory disorder that causes tumefactive lesions with a dense lymphoplasmacytic infiltrate rich in IgG4 plasma cells and storiform-pattern fibrosis. The clinical symptoms are relatively mild, and the condition is usually recognized by organ swelling and damage. When referring to the ophthalmic manifestations of IgG4-RD, the term IgG4-related ophthalmic disease (IgG4-ROD) is used. IgG4-ROD is characterized by bilateral lacrimal gland enlargement accompanied by 3 distinctive features: infraorbital nerve enlargement, extraocular myositis, and compressive optic neuropathy. IgG4 implies an underlying systemic disease process requiring evaluation to detect other systemic involvement. This includes hypophysitis and hypertrophic pachymeningitis, entities of neuro-ophthalmic interest. IgG4-ROD usually responds favorably to systemic corticosteroids but may be complicated by relapse during steroid taper. Rituximab has been shown to be effective for controlling steroid-refractory IgG4-RD. In contrast to IgG4-RD, an increasing number of cases of extranodal marginal B-cell lymphoma (MALT type) associated with IgG4-ROD have been described. IgG4 may be a risk factor for later emergence of low-grade B-cell lymphoma.

Ann Am Thorac Soc. 2014 Nov;11(9):1466-75.

Clinical review of pulmonary manifestations of IgG4-related disease.

Campbell SN1, Rubio E, Loschner AL.

Abstract

IgG4-related disease (IgG4-RD) is a recently recognized systemic disease characterized by tumefactive lesions in various organ systems. The list of organs that can be involved continues to expand, and recently computed tomography (CT) descriptions of the pulmonary lesions found in the disease have been described. The clinical symptoms are nonspecific and may include cough, dyspnea, chest pain, and fever. The appropriate clinical presentation along with elevated serum IgG4 concentrations and pathologic evidence of lymphoplasmacytic infiltrates with abundant IgG4-positive plasma cells and storiform fibrosis is consistent with the disease. Steroids are used to treat this disease in addition to immunosupressives such as cyclosporine or rituxumab for steroid refractory disease. The pulmonary manifestations and imaging features can often mimic malignancy, and as such knowledge of the diagnostic, clinicopathologic, and radiographic features of the disease is required in order to provide appropriate diagnostic workup and treatment.

Thank you very much, Dr.Safdar for very helpful references!

Best regards,

Wisit

My Pleasure

Best,

A

Hi Wisit

Yes. B-lymphocyte depletion with rituximab is now regarded as an effective therapeutic strategy for IgG4 related systemic disease, Please see the following aricles that may help ypu:

http://www.ncbi.nlm.nih.gov/pubmed/22210556

http://www.ncbi.nlm.nih.gov/pubmed/20191576

http://www.ncbi.nlm.nih.gov/pubmed/23653939

Thank you very much, Dr.Isho! 

These references are helpful!

Best regards,

Wisit