Author's Accepted Manuscript

‘Scleroderma renal crisis’ Nilanjana Bose MD, Andres Chiesa-Vottero MD, Soumya Chatterjee MD, MS, FRCP

www.elsevier.com/locate/semarthrit

PII: DOI: Reference:

S0049-0172(14)00321-7 http://dx.doi.org/10.1016/j.semarthrit.2014.12.001 YSARH50880

To appear in:

Seminars in Arthritis and Rheumatism

Cite this article as: Nilanjana Bose MD, Andres Chiesa-Vottero MD, Soumya Chatterjee MD, MS, FRCP, ‘Scleroderma renal crisis’, Seminars in Arthritis and Rheumatism, http://dx.doi.org/10.1016/j.semarthrit.2014.12.001 This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting galley proof before it is published in its final citable form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.

Title Page (REVISED VERSION) Title: ‘Scleroderma Renal Crisis’

Authors: 1. Nilanjana Bose, MD 2. Andres Chiesa-Vottero, MD 3. Soumya Chatterjee, MD, MS, FRCP

Running head: Scleroderma renal crisis

Grants or other financial supporters of the study: None

Address for correspondence: Soumya Chatterjee, MD, MS, FRCP Staff, Department of Rheumatic and Immunologic Diseases, Orthopedics and Rheumatology Institute, Cleveland Clinic, 9500 Euclid Avenue, Desk A50, Cleveland, OH 44195 U.S.A. Phone: 216-444-9945 Fax: 216-445-7569 E-mail: [email protected]

Address for reprint requests: same as above.

Total word count for the manuscript (not including title, abstract, tables, figures, table and figure legends, acknowledgements, and references): 4619.

Author contributions: Drs. Bose and Chatterjee had full access to all of the data in the study and they both take responsibility for the integrity and accuracy of the data. Concept: Bose and Chatterjee. Acquisition of data: Bose and Chatterjee. Analysis and interpretation of data: Bose, Chiesa-Vottero and Chatterjee. Manuscript preparation: Bose, Chiesa-Vottero and Chatterjee.

1 Abstract:

Objectives: To discuss the pathophysiology, risk factors, clinical manifestations, diagnosis, treatment, prevention and outcomes of Scleroderma Renal Crisis (SRC), a serious yet potentially treatable complication of scleroderma (systemic sclerosis).

Methods: A PubMed search for articles published in the English language up until 2013 was conducted using the following key words: scleroderma, systemic sclerosis, scleroderma renal crisis, renal, treatment, and prognosis. Literature was carefully reviewed and different risk factors, treatment options, prognostic factors and survival data were assessed.

Results: SRC occurs in about 10% of all patients with scleroderma. It is characterized by malignant hypertension and progressive renal failure. Around 10% of SRC cases may present with normal blood pressure, termed normotensive renal crisis. The etiopathogenesis is presumed to be a series of insults to the kidneys resulting in endothelial injury, intimal proliferation and narrowing of renal arterioles leading to decreased blood flow, hyperplasia of the juxtaglomerular apparatus, hyperreninemia and accelerated hypertension. Risk factors include rapid skin thickening, use of certain medications such corticosteroids or cyclosporine, new onset microangiopathic hemolytic anemia and/or thrombocytopenia, cardiac complications (pericardial effusion, congestive heart failure, and/or arrhythmias), large joint contractures, and presence of anti-RNA Polymerase III antibody. Since the 1970s, with the advent of angiotensin converting enzyme (ACE) inhibitors, mortality associated with SRC decreased from 76% to 55 years [odds ratio (OR) 2.57], cardiac involvement (OR 2.27), tendon friction rubs (OR 2.13), gastrointestinal involvement (OR 1.83) and rapid STPR (OR 1.74), along with other factors such as male gender (OR 1.60) and anti-RNA polymerase III antibody (OR O.61) (2). Currently, the leading causes of death in scleroderma are pulmonary fibrosis and pulmonary arterial hypertension.

4

Scleroderma renal crisis (SRC) has remained one of the most dreaded complications of scleroderma. It is regarded as a medical emergency. The cumulative incidence ranges between 10-19% (3;4). However, since the 1970s, with the introduction of angiotensin converting enzyme (ACE) inhibitors, the mortality and morbidity from SRC has decreased significantly. The 10 year survival among scleroderma patients has improved from 52 to 65% and survival among SRC patients from 120% of upper limit of normal for the local laboratory; (f) proteinuria ≥ 2+ by dipstick and confirmed by spot urine protein : creatinine ratio ≥ upper limit of normal; (g) hematuria ≥ 2+ on dipstick or ≥ 10 red blood cells/high power of field (in the absence of menstruation); (h) platelet count

Scleroderma renal crisis.

To discuss the pathophysiology, risk factors, clinical manifestations, diagnosis, treatment, prevention, and outcomes of scleroderma renal crisis (SRC...
703KB Sizes 4 Downloads 7 Views