Articles for Clinicians

ANA and Antibody Series

Scleroderma ANA and Antibody Testing Basics

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This introductory article discusses issues related to ANA and antibody testing for patients who have or might have systemic sclerosis.  It includes a discussion of research that shows that current testing methods commonly used for ANA and antibody screening have a high likelihood of falsely indicating a negative ANA result because of missing key SSc-related antibodies, often leading to incorrect or delayed diagnosis and treatments.

How to Do Scleroderma ANA and Antibody Testing Correctly

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This article is a short, bullet point summary version of the information found in the Scleroderma ANA and Antibody Testing article.  It briefly covers the problems resulting from changes in ANA testing methodology and details how to most effectively do appropriate ANA and antibody testing to help in clinical diagnosis of patients that may have systemic sclerosis or a scleroderma overlap syndrome such as Mixed Connective Tissue Disorder.

Changes in ANA and Antibody Levels In Scleroderma

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This article was prompted by a question asked by a patient wondering why her ANA suddenly becoming negative after having been positive for many years.  It discusses the research on when and how ANA and antibody levels change over time and the clinical significance of these changes.

The Puzzle of ANA-Negative Systemic Scleroderma

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Research studies consistently show that between 5% and 10% of patients with clear systemic scleroderma symptoms repeatedly test negative for ANA when done by the “gold standard” IFA method.  This paper discusses this unusual situation.

ANA and Antibody Test: Why the Testing Lab Matters (Future Article)

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ANA and antibody testing for systemic sclerosis can be very challenging.  This article will explain why clinicians need to be very aware of how different internal and external reference labs perform tests such as ANA screening panels and why just ordering a “scleroderma antibody panel” may lead to inadequate testing that may lead to incorrect or delayed diagnoses.

Scl-70 (Topoisomerase) Antibody Testing: the “False Positive” Problem (Future Article)

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There is a significant issue that can occur when using ELISA or Multiplex testing for Scl-70 (Topoisomerase) antibodies, one of the three most common SSc-specific antibodies.  Scl-70 antibodies are typically linked to diffuse SSc, but because of testing methodology issues, it is fairly common to get reproducible false positive Scl-70 testing results that can lead to incorrect or delayed diagnosis.  This article discusses the background research on this important issue and suggests ways to ensure that Scl-70 antibody testing is done is reliable manner to prevent these issues.

Therapeutic Plasma Exchange Series

Therapeutic Plasma Exchange for the Treatment of Systemic Sclerosis: A Brief Overview for Clinicians

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This brief overview document is designed to give clinicians an overview of the published research on the use of therapeutic plasma exchange (TPE) as a treatment for systemic sclerosis.  It includes references to additional resources including a very long term case report where TPE was used as the sole systemic treatment and also links to a recently published comprehensive review article.  For clinicians who are considering trying TPE as a treatment option, it also includes links to a document that gives a suggested protocol for a one-year individual of TPE and a handout for patients that describes the TPE experience and helps to prepare patients for a successful experience when undergoing plasma exchange.

Video: Therapeutic Plasma Exchange for the Treatment of Systemic Sclerosis: A Guide for Clinicians

YouTube Video: TPE as a Treatment for SSc

This video is an overview of the research literature on therapeutic plasma exchange (TPE) as a treatment for systemic sclerosis. In addition to an overview of the published research on the use of TPE as a treatment for systemic sclerosis, it also discusses important topics such as safety, cost, venous access, and other issues that need to be considered when evaluating TPE as a potential treatment option.  We suggest watching this video before reading the full review paper published in the Journal of Scleroderma and Related Disorders, referenced below.

Therapeutic Plasma Exchange as a Treatment for Systemic Sclerosis: A Comprehensive Review and Analysis

JSRD: TPE / SSc Review

Our comprehensive review of the research literature on the use of therapeutic plasma exchange is published in the June 2018 issue of the Journal of Scleroderma and Related Disorders.  We reviewed 46 reviewed articles involving a total of 572 patients, of which 455 received TPE.  It is published Open Access and can be freely read/downloaded from the Journal of Scleroderma and Related Disorders website at the link shown above.

Some of the topics covered in this article include:

  • Efficacy of TPE as a treatment for SSc
  • Contraindications
  • TPE complications and safety
  • Vascular access
  • Cost

Suggested Protocol for a One-Year Trial of Therapeutic Plasma Exchange for Treating Systemic Sclerosis

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The purpose of this document is to provide background information for clinicians and patients who have reviewed the research literature on the use of therapeutic plasma exchange (TPE) to treat systemic sclerosis (SSc) and made the determination that this may be an appropriate treatment option.  This paper includes discussions of TPE safety, cost, and venous access as well as providing background information on TPE to guide the clinician.  While this is not intended to be a formal research proposal, it is hoped that by following a standardized protocol for administering TPE, as well as including a basic set of objective measures during the trial of TPE, we may be able to glean important information that can be used as the basis for future well-designed clinical trials of TPE for treating patients with SSc.

Therapeutic Plasma Exchange: A Guide for Newbies

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While therapeutic plasma exchange is a routine, safe procedure that is widely used to treat a number of disorders, especially neurological disorders, it is not common used in the treatment of rheumatic diseases.  This document is written for patients (and family members) who are about to start TPE and is designed to make the TPE experience as successful and comfortable as possible.  We recommend that clinicians also read this document in order to be able to work more effectively with patients who are about to start to individual trials of therapeutic plasma exchange.

Additional Articles

Understanding the 2013 Formal Diagnosis Criteria for Systemic Sclerosis

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In late 2013, the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR) approved a new set of diagnostic criteria for systemic sclerosis (SSc), replacing the older 1980 diagnostic criteria.  These new standards will improve clinical diagnosis of SSc, but it is very important to understand that the reason for developing these new classification criteria was “to develop a set of criteria that would enable identification of individuals with SSc for inclusion in clinical studies”, not for the purpose of normal diagnosis of patients in a clinical setting. This brief article explains how the 2013 Classification Criteria are intended to be used in a clinical setting

2017 EULAR Recommendations for the Treatment of Systemic Sclerosis

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The European League for Rheumatism (EULAR) periodically reviews the most research literature on the treatment of systemic sclerosis (SSc) and publishes updated treatment recommendations. This brief article summarizes the recommendations in a concise format.  Clinicians and other interested people should refer to the full article for more detailed information (a direct link to the full, open access, article is included).