Classic gout in Hageman factor (Factor XII) deficiency

Area of Science:

• Rheumatology
• Hematology
• Internal Medicine

Background:

• Gouty arthritis is a common inflammatory condition often associated with hyperuricemia.
• Hageman factor (coagulation factor XII) plays a role in the intrinsic pathway of coagulation and inflammation.
• The precise role of Hageman factor in the pathogenesis of gouty arthritis remains incompletely understood.

Observation:

• A 62-year-old male with a history of gout presented with acute hemiplegia.
• Laboratory findings revealed elevated serum uric acid (10.3 mg/dL) and a markedly prolonged partial thromboplastin time (198 s).
• The patient exhibited deficient Hageman factor (factor XII) activity and antigen (<1% of normal).

Findings:

• Synovial fluid aspiration from the inflamed knee showed urate crystals and leukocytes, consistent with gouty arthritis.
• Crucially, Hageman factor antigen was absent in the synovial fluid.
• Despite the absence of Hageman factor, the patient experienced an acute gouty arthritis episode.

Implications:

• The case challenges the established role of Hageman factor in the inflammatory cascade of gouty arthropathy.
• This suggests that alternative inflammatory pathways may be sufficient for the development of acute gout.
• Further research is warranted to elucidate the complete mechanisms underlying gout pathogenesis in the absence of Hageman factor.