Overview
Definition:
Pediatric Inflammatory Bowel Disease (IBD) flare refers to a period of active, symptomatic disease characterized by increased inflammation and severity in children with Crohn's disease or ulcerative colitis
Management decisions for a flare often involve choosing between initial therapy with corticosteroids or newer biologic agents, depending on disease severity, location, and prior treatment history.
Epidemiology:
Pediatric IBD incidence is rising globally, with approximately 20-25% of IBD cases diagnosed in childhood
Ulcerative colitis is more common in younger children, while Crohn's disease is more prevalent in adolescents
Flares are a common occurrence, significantly impacting quality of life and growth.
Clinical Significance:
Effective and timely management of pediatric IBD flares is crucial to achieve remission, prevent complications, promote normal growth and development, and improve long-term outcomes
The choice between steroids and biologics is a cornerstone of modern pediatric IBD management, with significant implications for efficacy, safety, and long-term disease control.
Clinical Presentation
Symptoms:
Abdominal pain, often crampy and intermittent
Diarrhea, which may be bloody or mucousy
Rectal bleeding
Fever, especially with more severe inflammation
Weight loss or failure to thrive
Fatigue and malaise
Nausea and vomiting
Anal or perianal disease, including fissures, fistulas, or abscesses in Crohn's disease.
Signs:
Abdominal tenderness, often localized or diffuse
Palpable abdominal masses, particularly in Crohn's disease
Perianal examination findings may include erythema, edema, fissures, fistulas, or perianal abscesses
Signs of malnutrition, such as decreased muscle mass and subcutaneous fat
Fever
Pallor, suggestive of anemia.
Diagnostic Criteria:
Diagnosis of pediatric IBD is based on a combination of clinical symptoms, endoscopic findings, histological evidence of inflammation, and exclusion of other causes
Flare assessment involves evaluating the severity of symptoms and objective markers of inflammation
Pediatric Crohn's Disease Activity Index (PCDAI) and Pediatric Ulcerative Colitis Activity Index (PUCAI) are commonly used to quantify disease activity.
Diagnostic Approach
History Taking:
Detailed history of presenting symptoms including onset, duration, frequency, and character of stool (bloody, mucousy)
Previous IBD diagnosis and treatment history
Family history of IBD or autoimmune diseases
Nutritional status and growth trajectory
Extraintestinal manifestations, such as joint pain, skin rashes, or eye inflammation.
Physical Examination:
Thorough abdominal examination for tenderness, distension, masses, and organomegaly
Careful perianal examination for signs of inflammation, fissures, or fistulas
Assessment of nutritional status and hydration
Evaluation for extraintestinal manifestations.
Investigations:
Complete blood count (CBC) to assess for anemia, leukocytosis, and thrombocytosis
Inflammatory markers: C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) are elevated during flares
Stool studies: Stool culture to rule out infectious etiologies, fecal calprotectin to assess intestinal inflammation
Electrolytes, renal, and liver function tests
Nutritional assessment: Vitamin levels (e.g., B12, D, iron)
Imaging: Abdominal ultrasound, MRI, or CT enterography to assess disease extent and complications, especially in Crohn's disease
Endoscopy: Colonoscopy with biopsies is essential for diagnosis and assessing severity
upper endoscopy and capsule endoscopy may be indicated in selected cases.
Differential Diagnosis:
Infectious gastroenteritis (bacterial, viral, parasitic)
Allergic colitis
Food protein-induced enterocolitis syndrome (FPIES)
Celiac disease
Irritable bowel syndrome
Meckel's diverticulitis
Appendicitis
Malignancy (rare in pediatrics).
Management
Initial Management:
The decision to use steroids or biologics as initial therapy for a flare depends on several factors: disease severity (mild, moderate, severe), disease location (ileal, colonic, pancolitis), presence of complications (strictures, fistulas, abscesses), and patient factors (previous treatment response, adherence).
Medical Management:
Corticosteroids: Prednisolone or budesonide are commonly used for remission induction in moderate to severe flares
Doses vary based on severity and weight
Typical oral prednisolone dose is 1-2 mg/kg/day, max 60 mg/day
Budesonide is preferred for ileocolonic Crohn's due to its targeted release and lower systemic side effects
Steroids are generally for short-term use (8-12 weeks)
Biologics: Anti-TNF agents (infliximab, adalimumab) or vedolizumab are increasingly used as first-line therapy for moderate to severe IBD, especially in Crohn's disease with high-risk features (e.g., perianal disease, extensive inflammation, growth failure) or for patients who have failed steroids
Dosing is weight-based and administered intravenously or subcutaneously.
Surgical Management:
Surgery is typically reserved for managing complications of IBD, such as bowel obstruction due to strictures, intractable fistulas, abscesses, or perforation, or for refractory disease despite medical therapy
It is not considered first-line treatment for flares themselves.
Supportive Care:
Nutritional support: Adequate caloric and protein intake is vital
may require enteral or parenteral nutrition in severe cases
Hydration and electrolyte correction
Pain management
Monitoring for growth and development
Psychological support for the child and family.
Steroids Vs Biologics Initiation
Steroid Indications:
Mild to moderate flares in ulcerative colitis or Crohn's disease where rapid symptom control is needed
As a bridge therapy before biologics are effective
Patients with contraindications to biologics or where rapid symptom relief is paramount.
Steroid Limitations:
Short-term efficacy only
high relapse rates upon discontinuation
Significant side effects including growth suppression, bone demineralization, mood changes, increased infection risk, and Cushingoid features
Do not alter the long-term disease course.
Biologic Indications:
Moderate to severe flares, especially in Crohn's disease with high-risk features
Patients refractory to corticosteroids
To achieve and maintain long-term remission
To prevent complications and promote normal growth
As primary therapy in selected cases with early aggressive disease.
Biologic Advantages:
More durable remission rates compared to steroids
Potential to alter disease trajectory and prevent complications
Better long-term safety profile for growth and bone health compared to chronic steroid use
Vedolizumab targets gut-specific inflammation, potentially reducing systemic side effects.
Timing Of Decision:
Decision should be made by a pediatric gastroenterologist based on a comprehensive assessment of disease severity, extent, complications, and patient-specific factors
Early consideration of biologics in high-risk patients can lead to better outcomes and avoid prolonged steroid exposure.
Complications
Early Complications:
Steroid side effects: hyperglycemia, hypertension, insomnia, mood lability, increased risk of infection
Biologic side effects: infusion/injection site reactions, opportunistic infections (rare), allergic reactions
Severe flares can lead to dehydration, electrolyte imbalances, toxic megacolon (UC), bowel obstruction, perforation, or abscess formation.
Late Complications:
Steroid-induced growth failure, osteoporosis, cataracts, adrenal suppression
Biologic-related: development of antibodies, loss of response, rare neurological events
Chronic disease complications: strictures, fistulas, malnutrition, impaired growth, increased risk of colorectal cancer (long-standing extensive colitis).
Prevention Strategies:
Judicious use of corticosteroids, aiming for shortest duration and lowest effective dose
Early introduction of steroid-sparing agents like biologics or immunomodulators
Close monitoring for side effects and growth
Prompt treatment of infections
Adequate nutritional support
Regular endoscopic surveillance in high-risk patients.
Prognosis
Factors Affecting Prognosis:
Age at diagnosis (younger age at diagnosis often associated with more aggressive disease)
Disease extent and location (Crohn's disease with small bowel involvement and perianal disease tends to be more severe)
Presence of extraintestinal manifestations
Response to initial therapy
Adherence to treatment
Development of complications
Genetic factors.
Outcomes:
With appropriate management, many children can achieve and maintain remission, allowing for normal growth and development and a good quality of life
However, IBD is a chronic condition with relapsing-remitting patterns
Long-term outcomes are significantly improved with early diagnosis and effective, often biologic-based, therapies that minimize steroid exposure.
Follow Up:
Regular follow-up with a pediatric gastroenterologist is essential, typically every 3-6 months, or more frequently during flares or active treatment
Monitoring includes clinical assessment, inflammatory markers, growth parameters, nutritional status, and periodic endoscopic evaluation to assess disease activity and response to therapy
Long-term monitoring for complications and potential drug-related side effects is also critical.
Key Points
Exam Focus:
DNB/NEET SS questions often focus on differentiating IBD flares from infectious diarrhea, understanding the indications for steroids vs
biologics, recognizing steroid side effects, and identifying high-risk features for early biologic initiation
Knowledge of pediatric scoring systems (PCDAI, PUCAI) is also important.
Clinical Pearls:
Always consider infectious etiologies in the differential diagnosis of bloody diarrhea in children
Steroids are for short-term symptom control and remission induction
they do not alter the disease course
Biologics offer more durable remission and prevent long-term complications
Early aggressive therapy with biologics in high-risk pediatric IBD patients can lead to superior long-term outcomes and avoid cumulative steroid toxicity.
Common Mistakes:
Over-reliance on steroids for long-term management
Delaying biologic therapy in moderate-to-severe or high-risk disease
Underestimating the impact of IBD on growth and development
Failing to consider infectious mimics of IBD flares
Inadequate monitoring of growth and drug-related side effects.