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IgG4-related disease (IgG4-RD) is a recently described systemic entity of unknown origin. It predominantly affects older men and has distinctive histopathologic features as storiform fibrosis, obliterative phlebitis, dense lymphoplasmacytic infiltrate with immunostaining for IgG4, and it may be
IgG4-related disease (IgG4-RD) is an uncommon immune-mediated condition considered to be a systemic disease, described in multiple organ systems. IgG4-RD that involves the maxillary and sinonasal region is rare. This report presents a very rare presentation of IgG4-RD in the maxillary alveolar
BACKGROUND
Giant cell arteritis is the most common form of large-vessel vasculitides. However, it is probable that extracranial involvement is underdiagnosed in patients with classical giant cell arteritis. In the recent literature most cases of giant cell arteritis have been described in
A 66-year old man, who had been diagnosed with dilated cardiomyopathy and felt a progressive shortness of breath and fatigability, was admitted to hospital. Computed tomography showed a thickening of the aortic wall from the aortic arch to the aortic bifurcation, as well as mild pleural and
Emphasis is placed on the minor degree of caliectasis often observed in the presence of severe renal failure in patients with retroperitoneal fibrosis. This finding should suggest the diagnosis in individuals presenting with obscure causes of renal failure. A possible explanation for this is based
We report a patient of 32-year-old female with central IgG4-related disease. She developed headache and visual disturbance. On examination, she revealed diabetes insipidus, retrobulbar neuritis, hyperreflexia and limb weakness. Her laboratory findings showed serum IgG4 elevation, pleocytosis and
Retroperitoneal fibrosis is a rare cause of renal artery thrombosis, being the origin of renovascular hypertension, especially in patients less than thirty years old female, without particular medical history. A clinical case we present is a young 24 years old girl with no past history, hospitalized
We report a case of a 43-year-old female who presented with right ear fullness and otorrhea. She was initially diagnosed with mastoiditis that was not responsive to multiple courses of antibiotics and steroids. She was then diagnosed with refractory inflammatory pseudotumor, and subsequent
BACKGROUND
IgG4-related disease is a recently described multisystemic clinical entity that can occur with different clinical manifestations. The most often affected organs are the pancreas, bile duct and salivary glands, with unusual central nervous system affection.
METHODS
A 33 year old woman who
IgG4-related disease is a newly recognized systemic fibroinflammatory disorder. We report a 36-year-old man who presented with intractable right nasal pain and frontal headache for 1 month. Computed tomography revealed an ill-defined lesion with bony erosion over the right anterior ethmoid sinus and
Hypertrophic pachymeningitis is a rare disease, and the fibrosing inflammatory process causes a thickening of the dura mater. A 62-year-old male undergoing corticosteroid therapy for autoimmune pancreatitis presented with headache and right facial numbness. Brain CT and MRI revealed thickened mass
OBJECTIVE
Rosai-Dorfman disease is a rare entity that has been described as lymphadenopathy in young patients. Extranodal forms of this disease have been previously observed. The etiology of Rosai-Dorfman disease remains unknown, relationships with the IgG4-related sclerotic disease have been