Undifferentiated Connective Tissue Disease (What is the relationship between UCTD and SLE?) R. John Looney, MD Stephen and Elise Rosenfeld Distinguished Professor of Allergy and Clinical Immunology

Looking for Lupus

Copyright © American College of Rheumatology Slide Collection. All rights reserved. Red blood cell cast, urine

Copyright © American College of Rheumatology Slide Collection. All rights reserved. Systemic lupus erythematosus: brain (MRI)

M. Petri, et al. Arthritis and Rheumatism 64: 2677–2686, 2012 Sensitivity and specificity of each criterion for SLE

When do you call it lupus?

What difference does it make?

JM Roberson, et al. Rheum Dis Clin N Am 40 (2014) 621–635

Z Liu, et al. Nat. Med. 18; , 2012

Arbuckle NEJM 2003

Preclinical lupus thus encompasses a broad range of individuals, from individuals with enhanced genetic risk for SLE development without current clinical symptoms to individuals with autoantibodies and some clinical features of SLE that do not meet ACR disease classification criteria. Latent lupus identifies a group of individuals with features consistent with SLE that meet one or two of the ACR classification criteria along with the presence of minor criteria such as fever, fatigue, or lymphadenopathy. Incomplete lupus (ILE) refers to individuals with fewer than four of the ACR SLE classification criteria. JM Roberson, et al. Rheum Dis Clin N Am 40 (2014) 621–635 Preclinical lupus Latent Lupus Incomplete Lupus

JM Roberson, et al. Rheum Dis Clin N Am 40 (2014) 621–635 ILE 223 SLE 43 19%

Undifferentiated connective tissue disease (UCTD) is a broader term referring to clinical symptom manifestations suggestive of a specific connective tissue disease without meeting disease classification criteria. JM Roberson, et al. Rheum Dis Clin N Am 40 (2014) 621–635 Undifferentiated Systemic Rheumatic Disease (Undifferentiated Connective Tissue Disease ) 1.Raynauds 2.Skin rash 3.Inflammatory arthritis

Undifferentiated Systemic Rheumatic Disease (Undifferentiated Connective Tissue Disease ) What happens over 10 years? 1.60% remain USRD 2.30% meet criteria 3.10% complete remission

JM Roberson, et al. Rheum Dis Clin N Am 40 (2014) 621– UCTD 115 SLE 10%

Can we prevent lupus? R. John Looney, MD Stephen and Elise Rosenfeld Distinguished Professor of Allergy and Clinical Immunology

JM Roberson, et al. Rheum Dis Clin N Am 40 (2014) 621–635

EC Somers, et al. Lupus (2014) 23, 568–576

A Finckh, et al. Rheum Dis Clin N Am 40 (2014) 771–785 Can we prevent lupus?

A Finckh, et al. Rheum Dis Clin N Am 40 (2014) 771–

Triggering Clinical Disease In SLE is often availability of antigen Exposure to UV light damages keratinocytes releasing autoantigens Viral infections or adverse reaction to drugs Breakdown of the blood-brain barrier allows anti-neuronal antibodies to enter the CNS

How does smoking affect people with lupus? Smoking or being around people who smoke may increase your risk of pneumococcal pneumonia. If you smoke and have lupus you greatly increase the risk of damaging your heart. Smoking can magnify the effect of Raynaud's Smoking nworsens peripheral vascular disease. Smoking increases the risk of stroke. Smokers progressed to end-stage kidney disease far more quickly than did non- smokers. Smoking can contribute to common disorders as heartburn. Lupus skin disease is more active in smokers than non-smokers. Studies in mice have linked smoking hair loss. Smoking has been shown to interfere with the benefits of drugs such as hydroxychloroquine Smoking slows bone healing More than any other lifestyle choice you make, quitting cigarettes will have the greatest positive impact on your lupus!

JA James, et al. Lupus (2007) 16, 401–409

P = P = P = 0.03 NS

NEJM 347: , 2002