Villonodular synovitis (pigmented)


pigmented villonodular synovitis: Pigmented villonodular synovitis (PVNS) is a rare benign proliferative disorder of the synovial membrane primarily affecting young adults (with a peak age of onset in the second to fourth decade of life) characterized by proliferative, locally invasive tumor-like lesions, usually involving a single joint, tendon sheath or bursae (most commonly the joints of the knee and hip and rarely others such as the ankle, shoulder and temporomandibular joints). It presents with pain and limitation of motion along with swelling, heat and tenderness over the involved joint, eventually leading to arthritic degeneration and significant locomotor deficit, if left untreated. PVNS can recur in patients even after treatment.

Data source
FinnGen phenocode M13_VILLONODULAR
Hospital Discharge registry ICD-10: M122
Cause of Death registry ICD-10: M122
Level in the ICD-hierarchy 4
First defined in version DF2
DOID 2702, 9898
GWAS catalog 1001106
MESH D013586
SNOMED CT 95412009

Key figures

Sex All Female Male
Number of individuals 57 41 16
Unadjusted prevalence (%) 0.04 0.05 0.03
Mean age at first event (years) 41.68 39.96 46.09
Median number of events / individual 1 1 1

Clinical metrics

Sex All Female Male
Recurrence within 6 months (%) 10.53 7.32 18.75
Case fatality at 5-years (%) 0.0 0.0 0.0