Immunoglobulin G4-related disease (IgG4-RD) is an immunemediated fibro-inflammatory condition that is characterized by extensive storiform fibrosis, obliterative phlebitis, thick lymphoplasmacytic infiltrates with a high percentage of IgG4-bearing plasma cells, and frequent tissue eosinophilia. 1 This disorder, which can affect practically any organ synchronously or metachronously, can result in the development of sclerotic masses of varying sizes.Differentiating IgG4-RD from other rheumatic diseases is challenging for clinicians. Currently, IgG4-RD is recognized as an important cause of idiopathic orbital inflammation (IOI) which must be differentiated from other pathologies that include Hodgkin's lymphoma and non-Hodgkin's lymphoma, granulomatosis with polyangiitis (Wegener's), Graves' orbitopathy, and other conditions. [1][2][3][4] Their incidence was estimated at 4.7%-6.3% of all orbital diseases. 5 We report a case of multi-organ IgG4-RD revealed by an orbital IOI.

Agranulocytosis is a rare acute condition characterized by severe a < gft (neutropenia in which the neutrophils count is less than 100/mm 3 . It can be classified into two categories, inherited, and acquired. Acquired agranulocytosis is not commonly caused by auto‐immune diseases such as systemic lupus erythematosus (SLE). We report a case of a patient suffering from agranulocytosis related to SLE at disease onset, associated with other rare disease involvements.

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