Pediatrics · MU Sofia

26. Connective tissue diseases in children.

Covers the main connective tissue diseases seen in children: juvenile lupus, juvenile dermatomyositis, localised and systemic scleroderma, and rarer conditions such as mixed connective tissue disease, Sjögren syndrome and antiphospholipid syndrome. It sets out organ features, laboratory tests and treatment for each, with a table of lupus features by organ system, a table of scleroderma types, and a closing section on oral and dental findings.

Inside: 5 sections · 2 tables · 16 flashcards · 4 min

Topic 26 of 30 in the MU Sofia syllabus for Pediatrics (Medicine, Year 5). The free AceMed note covers it in 5 sections, about 449 words (4 min of reading), with 16 flashcards.

In this note

  1. Introduction
  2. Juvenile systemic lupus erythematosus (jSLE)
  3. Juvenile dermatomyositis (JDM)
  4. Scleroderma
  5. Other conditions

Introduction

Connective tissue diseases are systemic autoimmune diseases with autoantibodies that attack skin, joints, vessels and internal organs. In children the main ones are juvenile systemic lupus erythematosus, juvenile dermatomyositis and scleroderma (mostly localised); mixed connective tissue disease and Sjögren syndrome are rare. They are often more severe in children than in adults.

Juvenile systemic lupus erythematosus (jSLE)

Lupus starting before 18 years (15–20% of all lupus), mostly teenage girls. Immune complexes and autoantibodies cause inflammation in many organs. Compared with adults, children have more kidney, brain and blood involvement.

Tests: ANA (positive in almost all; the entry criterion at ≥ 1:80, EULAR/ACR 2019), anti-dsDNA (activity, nephritis) and anti-Sm (specific), low C3 and C4, antiphospholipid antibodies, blood count, urine protein, kidney function.

Treatment

  • Hydroxychloroquine for everyone (yearly eye checks); sun protection.
  • Corticosteroids (pulses for severe disease), then steroid-sparing drugs: azathioprine, mycophenolate (nephritis), methotrexate (skin and joints), cyclophosphamide (severe), rituximab, belimumab.
  • Blood pressure control, bone health, vaccinations, attention to growth, mental health and adherence.

Neonatal lupus: maternal anti-Ro/La antibodies cross the placenta: congenital complete heart block (permanent; may need a pacemaker), annular rash, low blood counts, liver changes (transient).

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