Severe headache complicated by vertical atlantoaxial subluxation in diffuse systemic sclerosis with crowned dens...

Area of Science:

• Rheumatology
• Neurology
• Radiology

Background:

• Diffuse systemic sclerosis (SSc) is a multisystem autoimmune disease characterized by fibrosis, vascular abnormalities, and immune dysregulation.
• Ectopic calcification is a known, though not fully understood, complication in SSc.
• Atlantoaxial subluxation (AAS) is a rare but serious condition affecting the upper cervical spine.

Observation:

• A 34-year-old female patient with diffuse SSc and generalized ectopic calcification presented with severe headache.
• Imaging revealed vertical atlantoaxial subluxation (AAS).
• Calcification around the odontoid process, consistent with a 'crowned dens' pattern, was noted.

Findings:

• The case highlights a rare presentation of AAS in a patient with diffuse systemic sclerosis.
• The presence of extensive ectopic calcification in SSc may predispose to or be associated with spinal complications like AAS.
• Severe headache in SSc patients with widespread calcification warrants investigation for AAS.

Implications:

• AAS should be considered in the differential diagnosis of severe headache in patients with systemic sclerosis, particularly those with extensive ectopic calcification.
• This case underscores the importance of thorough neurological and radiological evaluation in SSc patients presenting with atypical symptoms.
• Further research may elucidate the specific mechanisms linking SSc, ectopic calcification, and cervical spine instability.