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Open Access Review

Renal involvement in autoimmune connective tissue diseases

Andreas Kronbichler and Gert Mayer*

Author affiliations

Department of Internal Medicine IV, Nephrology and Hypertension, Medical University Innsbruck, Anichstraße 35, Innsbruck, 6020, Austria

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Citation and License

BMC Medicine 2013, 11:95  doi:10.1186/1741-7015-11-95

Published: 4 April 2013

Abstract

Connective tissue diseases (CTDs) are a heterogeneous group of disorders that share certain clinical presentations and a disturbed immunoregulation, leading to autoantibody production. Subclinical or overt renal manifestations are frequently observed and complicate the clinical course of CTDs. Alterations of kidney function in Sjögren syndrome, systemic scleroderma (SSc), auto-immune myopathies (dermatomyositis and polymyositis), systemic lupus erythematosus (SLE), antiphospholipid syndrome nephropathy (APSN) as well as rheumatoid arthritis (RA) are frequently present and physicians should be aware of that.

In SLE, renal prognosis significantly improved based on specific classification and treatment strategies adjusted to kidney biopsy findings. Patients with scleroderma renal crisis (SRC), which is usually characterized by severe hypertension, progressive decline of renal function and thrombotic microangiopathy, show a significant benefit of early angiotensin-converting-enzyme (ACE) inhibitor use in particular and strict blood pressure control in general. Treatment of the underlying autoimmune disorder or discontinuation of specific therapeutic agents improves kidney function in most patients with Sjögren syndrome, auto-immune myopathies, APSN and RA.

In this review we focus on impairment of renal function in relation to underlying disease or adverse drug effects and implications on treatment decisions.

Keywords:
Renal involvement; Connective tissue diseases; Sjögren syndrome; Scleroderma renal crisis, Dermatomyositis/polymyositis; Systemic lupus erythematosus; Antiphospholipid syndrome; Rheumatoid arthritis