Can Autoimmune Diseases Cause Chills?
Yes, certain autoimmune diseases, particularly autoinflammatory syndromes, commonly cause chills as part of their febrile episodes, though chills are not a universal feature of all autoimmune conditions.
Autoinflammatory Diseases and Chills
The most direct connection between autoimmune/autoinflammatory conditions and chills occurs in the autoinflammatory syndromes, where recurrent fever is a cardinal feature:
Cryopyrin-associated periodic syndromes (CAPS) present with fever and systemic inflammation, often triggered by cold exposure in milder forms like familial cold autoinflammatory syndrome (FCAS), where cold-induced flares typically last less than 24 hours 1
Tumor necrosis factor receptor-associated periodic syndrome (TRAPS) manifests with periodic fever attacks associated with migratory rash, periorbital edema, and various pain syndromes 1
Mevalonate kinase deficiency (MKD) causes recurrent fever episodes lasting 4-6 days with gastrointestinal symptoms, rash, and lymphadenopathy 1
Deficiency of IL-1 receptor antagonist (DIRA) presents with fever alongside pustular skin disease and bone involvement 1
Systemic Lupus Erythematosus and Fever
Fever is a recognized component of SLE classification criteria and occurs in up to 90% of patients during disease activity 1, 2:
Fever in SLE is often associated with systemic symptoms including chills, poor appetite, and weight loss 1
The critical clinical challenge is differentiating fever from SLE disease activity versus infectious causes, as both can present similarly 2
SLE patients have increased mortality risk from infections, making this distinction crucial for management 1, 2
Immune Checkpoint Inhibitor-Related Autoimmunity
Cancer immunotherapy can trigger autoimmune phenomena that include systemic symptoms:
Rheumatic immune-related adverse events from checkpoint inhibitors may present with fever, fatigue, myalgia, and arthralgia 1
Vasculitis induced by these agents can manifest with fever, fatigue, and various systemic symptoms 1
Clinical Approach to Fever in Autoimmune Disease
When evaluating fever and chills in the context of known or suspected autoimmune disease, prioritize:
Distinguish infection from disease activity - This is the most critical determination, as therapeutic approaches are contradictory (immunosuppression vs. antimicrobials) 2
Assess for autoinflammatory syndrome features 1:
- Pattern of fever (periodic vs. continuous)
- Associated rash (urticarial, migratory, pustular)
- Organ-specific symptoms (joint pain, abdominal pain, neurologic symptoms)
- Cold-triggered symptoms
- Family history
Monitor inflammatory markers - ESR, CRP, and serum amyloid A (SAA) should be assessed at each visit for autoinflammatory diseases 1
Evaluate for complications - Screen for hepatosplenomegaly, lymphadenopathy, and AA amyloidosis with urinalysis every 6-12 months 1
Important Caveats
Fever may be absent in elderly patients, after antibiotic pretreatment, in immunocompromised patients, or with less virulent organisms 1
Recurrent fever requires broad differential diagnosis including malignancies, infections, and other inflammatory conditions before attributing to autoinflammatory disease 1, 3
Autoantibodies may be absent in many autoinflammatory conditions, unlike classic autoimmune diseases 1
The presence of fever with chills in autoimmune disease warrants urgent evaluation to exclude life-threatening infections, particularly in patients on immunosuppressive therapy 1, 2