Collagen Vascular Diseases: SLE, Dermatomyositis, Scleroderma, and MCTD

Richard K. Vehe; Mona M. Riskalla

doi:10.1542/pir.2017-0262

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Richard K. Vehe, MD*
Mona M. Riskalla, MD*

^*Division of Pediatric Rheumatology, Department of Pediatrics, University of Minnesota and the University of Minnesota Masonic Children’s Hospital, Minneapolis, MN

AUTHOR DISCLOSURE
Drs Vehe and Riskalla have disclosed no financial relationships relevant to this article. This commentary does not contain a discussion of an unapproved/investigative use of a commercial product/device.

Abbreviations:

ANA:: antinuclear antibody
CTD:: connective tissue disease
CVD:: collagen vascular disease
dsDNA:: doubled-stranded DNA
DM:: dermatomyositis
ENA:: extractable nuclear antigen
JIA:: juvenile idiopathic arthritis
MCTD:: mixed connective tissue disease
NFC:: nail fold capillary
RA:: rheumatoid arthritis
RF:: rheumatoid factor
RP:: Raynaud phenomenon
RNP:: ribonucleoprotein
SLE:: systemic lupus erythematosus

Practice Gap

Timely and accurate recognition of collagen vascular disorders (CVDs), and implementation of effective screening and referral processes for patients suspected of having a CVD, remain a challenge for many physicians. The result, too often, is unnecessary testing and referrals, and in some cases unnecessary anxiety for physicians, patients, and parents.

Objectives

After completing this article, readers should be able to:

Recognize the common clinical symptoms and signs of systemic lupus erythematosus, dermatomyositis, and scleroderma, and their distinction from common infectious mimics.
Recognize the testing that can clarify the likelihood of whether a child has a rheumatic disease, including the limited utility of early serologic testing for autoantibodies.
Recognize the prognosis and management objectives for these often-chronic disorders, and practical steps to ensure excellent outcomes.

Introduction

Timely and accurate recognition of collagen vascular diseases (CVDs), and implementation of effective screening and referral processes for patients suspected of having a CVD, remain challenging for many physicians. The result, too often, is unnecessary testing with questionable results, leading in some cases to unnecessary referrals and anxiety for physicians, patients, and parents. (1)

Sometimes, CVDs are also referred to as connective tissue diseases (CTDs), but neither name fully captures their essential nature. These are not primarily heritable disorders affecting collagen-containing structures, or disorders of vascular development, but rather are immune-mediated inflammatory diseases. The immunologic targets vary, but the inflammatory disease often affects collagen-containing tissues, including the musculoskeletal system and many other tissues. The targeting of blood vessels can lead to manifestations ranging from reversible vasospasm of Raynaud phenomenon (RP) to vascular injury of 1 or more organ systems, often with serious consequences.

The diseases that …