What diseases are associated with urticaria?

Diseases Associated with Urticaria

Urticaria is most strongly associated with thyroid autoimmunity (present in 14-20% of patients), followed by celiac disease in children, and autoimmune conditions including rheumatoid arthritis, systemic lupus erythematosus, and Sjögren syndrome, particularly in female patients. 1, 2, 3

Autoimmune and Endocrine Associations

Thyroid Disease

• Thyroid autoimmunity occurs in 14% of chronic urticaria patients compared to 6% in the general population, representing the most prevalent disease association 1
• Approximately 20% of chronic spontaneous urticaria patients have autoimmune thyroiditis 2
• Both hypothyroidism and hyperthyroidism show increased prevalence, along with antithyroid antibodies even in euthyroid individuals 1, 3
• The association with Hashimoto's thyroiditis and Graves' disease has been documented, with urticaria sometimes preceding thyroid disease diagnosis by over 1 year 4

Other Autoimmune Diseases

• Female patients with chronic urticaria have significantly higher incidence of multiple autoimmune conditions, typically diagnosed within 10 years after urticaria onset 3
• Rheumatoid arthritis shows strong association, particularly in female patients 3
• Systemic lupus erythematosus is more prevalent in urticaria patients, with leucopenia potentially detected on screening 1, 3
• Sjögren syndrome demonstrates increased incidence in female chronic urticaria patients 5, 3
• Type 1 diabetes mellitus occurs more frequently in female patients with chronic urticaria 5, 3

Gastrointestinal Associations

Celiac Disease

• A significantly higher prevalence of celiac disease exists in children and adolescents with severe chronic urticaria compared to case-matched controls 1
• This association is particularly important in pediatric populations with refractory urticaria 5, 3

Helicobacter pylori

• Meta-analysis demonstrates that chronic urticaria resolution is more likely when H. pylori eradication is successful (Quality of evidence I, Strength of recommendation B) 1
• This represents one of the few infectious associations with substantial evidence support 1

Infectious Associations

Limited Evidence for Occult Infections

• Associations with dental abscess and gastrointestinal candidiasis have been proposed but have little supporting evidence (Quality of evidence III) 1, 6
• Bowel helminth infections may present with eosinophilia on screening blood work 1

Vasculitic and Inflammatory Conditions

Urticarial Vasculitis

• Urticarial vasculitis represents a distinct entity requiring lesional skin biopsy for diagnosis, showing leucocytoclasia, endothelial damage, and perivascular fibrin deposition 1
• Hypocomplementaemic disease (low C3 and C4) carries a worse prognosis than normocomplementaemic disease 1
• ESR is typically raised in urticarial vasculitis, distinguishing it from ordinary chronic urticaria 1

Autoinflammatory Syndromes

• ESR is always raised in autoinflammatory syndromes, providing a screening marker 1

Metabolic and Psychiatric Comorbidities

• Metabolic syndrome affects 6-20% of chronic spontaneous urticaria patients 2
• Anxiety occurs in 10-31% and depression in 7-29% of patients, representing significant psychiatric burden 2
• These comorbidities contribute substantially to impaired quality of life 2

Important Negative Associations

Malignancy

• No statistical association exists between malignancy and urticaria (Quality of evidence II-ii), despite individual case reports 1, 6
• Routine cancer screening beyond age-appropriate guidelines is not indicated 1

Clinical Implications for Screening

For chronic urticaria nonresponders with severe disease, screening should include: 1

• Thyroid autoantibodies and thyroid function tests (highest yield)
• Full blood count with differential (to detect eosinophilia, leucopenia)
• Erythrocyte sedimentation rate (normal in ordinary chronic urticaria, raised in vasculitis and autoinflammatory conditions)
• Consider celiac screening in children and adolescents with severe disease
• Autologous serum skin test (ASST) in specialized centers for autoimmune urticaria

Common pitfall: Extensive investigation is not required for mild chronic urticaria responding to H1 antihistamines—investigations should be guided by clinical severity and treatment response 1