Abstract

CORREA LEMOS, María Eugenia; SAGER, Lorena Ayelén; ORTIZ, Alberto  and  PAIRA, Sergio. Patients with orbitary and kidney commitment: Rare differential diagnoses. Rev. argent. reumatolg. [online]. 2021, vol.32, n.4, pp.28-37. ISSN 0327-4411.

A series of three cases that consulted the rheumatology service due to orbital and renal involvement is reported. One of them presented orbital pseudotumor with proteinuria in the nephrotic range, a biopsy was performed, finding dense lymphoplasmacytic infiltrate and storiform fibrosis with immunohistochemistry: 15 IgG4 positive cells per HPF and IgG/IgG4 ratio ≤40%, concluding diagnosis of IgG4 related disease. The second and third cases presented ocular involvement with raccoon eyes and yellowish lesions on the eyelids, both with proteinuria greater than 500 mg/24 h, with apple-green birefringence of amyloid on congo red staining. Different rare differential diagnoses to take into account in these patients are discussed.

Keywords : amyloidosis; IgG4-related disease; sarcoidosis; orbitopathy.

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