#77: Childhood-Onset SLE – Is It Sometimes Lupus?

February 1, 2023 | By Sam Masur

Summary

Despite the catch phrase of a certain fictional physician, sometimes it really is Lupus! Dr. Rebecca Sadun (Duke) teaches us the fundamentals of how to recognize, evaluate and treat this complex disease.

Lupus Pearls

1. The various classification criteria for Systemic Lupus Erythematosus (SLE) are helpful for guiding initial history taking, physical examination and laboratory evaluation when there is suspicion for lupus.
2. A positive ANA is highly sensitive but not at all specific for SLE.
3. A mainstay of preventing lupus flares is hydroxychloroquine, which should be continued lifelong in all patients without contraindications.
4. There are many different DMARDs (Disease-Modifying Antirheumatic Drugs), the three most commonly used for managing pediatric lupus are methotrexate, mycophenolate and azathioprine.
5. You will impress your local rheumatologist if you calculate a SLEDAI (SLE Disease Activity Index) score for your lupus patients!

Lupus Show Notes

Epidemiology of pediatric lupus

• SLE is rare in children before the age of 9 years, and the incidence increases as age advances with the highest incidence of pediatric lupus occurring in older adolescents 
• The ratio of lupus in girls to boys is 1:3 prior to puberty, 9:1 during puberty and 5:1 after puberty

Clinical presentation 

• Highly heterogenous clinical presentation
• Fatigue, fevers, weight loss and joint pain and stiffness are common presenting symptoms
• Expert tip: duration of symptoms is important, Dr. Sadun advises that symptoms lasting hours to days are not concerning for lupus, however symptoms lasting weeks to months may be
• Mucocutaneous manifestations are present in 2/3 of children with lupus, examples include malar and discoid rashes, alopecia, and oral or nasal ulcers
• Expert tip: oral and nasal ulcers caused by lupus are painless thus patients are unlikely to report them; Dr. Sadun recommends examining the nasal septum and hard palate, and notes that they may appear as areas of hyperemia, pallor or even petechiae 

Diagnosis

• The 2012 SLICC (Systemic Lupus International Collaborating Clinics) classification criteria, 1997 ACR (American College of Rheumatology) criteria and 2019 EULAR/ACR (European Alliance of Associations for Rheumatology/ACR) criteria are all helpful for guiding diagnosis of SLE
• Expert tip: Dr Sadun recommends referring to any of the above criteria and notes that the 2012 SLICC criteria are most commonly used in current clinical practice for pediatric lupus
• Clinical criterion for diagnosis includes the presence of characteristic:

1. 1. Mucocutaneous manifestations
   2. Serositis
   3. Joint disease
   4. Cytopenias or hemolytic anemia 
   5. Renal involvement 
   6. CNS involvement

• Lab tests important for establishing a diagnosis of SLE include:
  • a) ANA
  • b) Anti-dsDNA
  • c) Anti-Sm
  • d) C3 and C4
  • e) Antiphospholipid autoantibodies

• Expert tip: 10-15% of health children will have a positive ANA, Dr. Sadun advises against ordering this when the index of suspicion is low

Treatment

• All patients should be treated with hydroxychloroquine to prevent flares unless unable to tolerate
• Glucocorticoids may be required to induce remission initially or manage flares
• DMARDs used in the treatment of SLE include methotrexate, mycophenolate and  azathioprine
• Choice of DMARD is guided by symptoms and organ involvement; for instance cyclophosphamide is used if CNS involvement is present
• Medications other than hydroxychloroquine are tapered and stopped if possible

Other Stuff

Dr Sadun recommends checking out videos of the pGALS (Pediatric Gait Arms Legs and Spine) exam for a quick and effective way to do an MSK physical examination!

Goal

Listeners will learn what clinical presentations should prompt suspicion for SLE, how to initiate diagnostic evaluation and gain an understanding of the fundamentals of management.

Learning Objectives

After listening to this episode listeners will…  

1. Recognize the importance of thorough history taking and physical examination in the evaluation of SLE
2. Be familiar with how to initiate laboratory evaluation for suspected SLE
3. Explain the role of hydroxychloroquine, glucocorticoids and DMARDs in the treatment of SLE
4. Describe common and severe manifestations of SLE by organ system

Disclosures

Dr. Rebecca Sadun reports no relevant financial disclosures. The Cribsiders report no relevant financial disclosures.

Citation

Chisholm J, Sadun R, Cruz M, Masur S, Berk J. “77 Childhood-Onset SLE: Is It Sometimes Lupus?”. The Cribsiders Pediatric Podcast. https:/www.thecribsiders.com/ February 1, 2023.