Presentation: Intraspinal and Paraspinal Calcinosis Associated with Systemic Sclerosis (2007)

8 Intraspinal and Paraspinal Calcinosis Associated with Systemic Sclerosis

BACKGROUND: Although calcinosis in the extremities is known as a common manifestation in patients with systemic sclerosis (SSc), calcinosis in the thoracic spine has been rarely reported in SSc. We encountered a woman with the diffuse cutaneous form of SSc who presented a rapidly progressing muscle weakness and sensory disturbance in her lower extremities. CT of her thoracic spine showed massive intraspinal calcinosis (ISC) and paraspinal calcinosis (PSC), causing spinal cord compression. To clarify the frequency of ISC and PSC and disease-phenotype associations, we conducted CT of the chest in patients with SSc.
METHODS: CT of the chest was performed in 41 patients with SSc (36 females, 5 males; mean age 60.3 ± 13.3 years) and images were interpreted blindly, together with those of 33 patients with other connective tissue diseases including rheumatoid arthritis (RA) (n=26) and systemic lupus erythematosus (SLE) (n=14). Incidence of intracranial calcinosis was also investigated using CT of the brain. The patients' medical records were reviewed for clinical manifestations and laboratory data.
RESULTS: PSC in the thoracic spine was found in 24 of 41 patients (58.5%), and 12 of these 24 patients showed ISC simultaneously. On the contrary, among the patients with RA and SLE, only one patient with RA showed PSC (p<0.0005 for RA vs. SSc, and p=0.005 for SLE vs. SSc) and none of them showed ISC (p=0.002 for RA vs. SSc, and p=0.02 for SLE vs. SSc). Two SSc patients with PSC and ISC showed serious weakness and sensory disturbance of lower extremities. The prevalence of intracranial calcification was similar in patients with SSc, RA and SLE patients (27%, 23% and 21%, respectively) and was not correlated with the presence of PSC or ISC. PSC and ISC were closely associated with calcinosis cutis (p=0.015 for PSC and p=0.002 for ISC), regular occurrence of Raynaud’s phenomenon (p<0.0005 for PSC and p=0.006 for ISC) and digital ulcerations (p=0.008 for PSC and p=0.007 for ISC). The mean age, duration of SSc, severity of skin involvement or extent of the interstitial lung disease was not significantly different between SSc patients with and without spinal calcinosis. Similarly, no relationship was found between the presence of PSC or ISC and serum calcium level, serum cholesterol level, immunological features or medications.
CONCLUSIONS: PSC and ISC are closely associated with vascular complications in SSc, and may cause neurological manifestations crucially.

 T. Ogawa, None; K. Ogawa, None; T. Ogura, None; Y. Izumi, None; N. Hayashi, None; A. Hirata, None; R. Miura, None; E. Saito, None.