Introduction IgG4-related disease (IgG4-RD) is characterized by the growth of pseudotumors, which can affect almost every organ. Elevated serum IgG4 levels are present in only two-thirds of the patients and are not specific. Diagnosis is difficult and is usually based on a biopsy. Clinical case A 39-year-old man presented complaints of low back pain and fever. A renal computed tomography (CT) scan revealed a voluminous mass next to the right kidney and a biopsy showed an inflammatory process and excluded a neoplasm. A follow-up CT scan at four months revealed total regression without any treatment. Three years later, the patient presented with diplopia and right proptosis. MRI of the orbits revealed a retro-ocular mass; biopsy excluded a malignant process. Infectious, autoimmune or paraneoplastic diseases were also excluded. Although the patient's IgG4 serum levels were normal, histopathological reobservation of the renal biopsy revealed IgG4-positive plasma cells, thus confirming the diagnosis. The patient was prescribed a daily dose of 40 mg of prednisolone and regression of the right orbital lesion was observed. Discussion IgG4-RD is a rare and recently described condition. Most anatomic pathology laboratories do not routinely test for it. Spontaneous pseudotumor remission is possible, even when associated with the renal phenotype, although this has not been described until now.

Pedro Manuel Oliveira

Ana Filipa Geraldo

Tiago Pereira

Marta Barbedo

Acta reumatológica portuguesa

ACTA REUMATÓLOGICA PORTUGUESA 是一份科学同行评审期刊，涵盖风湿性疾病的各个方面以及与风湿病学相关的内容。该杂志发表原创文章、评论、临床病例、风湿病学图像、致编辑的信和临床教学（例如指南和临床协议）。自 1973 年出版以来，ACTA REUMATOLÓGICA PORTUGUESA 是葡萄牙风湿病学会的官方科学出版物，促进风湿病知识和调查以及风湿病学发展的非营利组织。

Acta Reumatologica Portuguesa is a peer-reviewed scientific journal that covers all aspects of rheumatic diseases and related topics. The journal publishes original research articles, reviews, clinical cases, rheumatology images, letters to the editor, and clinical education materials (such as guidelines and clinical protocols). Since its inception in 1973, Acta Reumatologica Portuguesa has been the official scientific publication of the Portuguese Society of Rheumatology, promoting the dissemination of knowledge and research in rheumatology as a non-profit organization.

《Acta Reumatologica Portuguesa》是一本经过同行评审的科学期刊，涵盖风湿性疾病及其相关领域的各个方面。该期刊发表原创研究文章、评论、临床病例、风湿病学图像、致编辑的信件以及临床教学内容（如指南和临床协议）。自1973年创刊以来，《Acta Reumatologica Portuguesa》一直是葡萄牙风湿病学会的官方科学出版物，致力于促进风湿病知识的传播和研究，以及风湿病学的发展。

Category	Quartile
医学	4
医学, 风湿病学	4

IgG4-related disease with renal and orbital involvement: a clinical case.

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