Pigmented villonodular synovitis
| Pigmented villonodular synovitis | |
|---|---|
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| Micrograph of pigmented villonodular synovitis. H&E stain. | |
| Classification and external resources | |
| Specialty | rheumatology |
| ICD-10 | M12.2 |
| ICD-9-CM | 719.20 |
| DiseasesDB | 29902 |
| MedlinePlus | 3 |
| eMedicine | article/394649 |
| MeSH | D013586 |
Pigmented villonodular synovitis (PVNS) is a joint disease characterized by inflammation and overgrowth of the joint lining. It usually affects the hip or knee. It can also occur in the shoulder, ankle, elbow, hand or foot. In PVNS the lining of the joint, called the synovium, becomes swollen and grows. This growth harms the bone next to the joint. The lining also makes extra fluid that can cause swelling and make movement painful. PVNS is idiopathic, it doesn't seem to run in families or be caused by certain jobs or activities. Surgery can help but, even with treatment, PVNS comes back about half the time. If the pain remains then radiation therapy may help. In the worst cases the joint must be replaced.
In general, pigmented villonodular synovitis often manifests initially as sudden onset, unexplained joint swelling and pain; the joint swelling is disproportionate to the amount of pain the patient feels at first. Decreased motion and increased pain occur as the disorder progresses as well as locking of the joint. The localized form often manifests initially as a painless, slow-growing mass and progresses to the other common symptoms of PVNS. The swelling often feels warm to the touch. Diffuse PVNS symptoms are often confused with those of Rheumatoid arthritis. While pigmented villonodular synovitis can occur in both pediatric and geriatric patients, it is more common with ages 20–50.
PVNS is locally aggressive and can spread to surrounding tissues, causing bone erosion and tissue damage. If not treated early, it can spread to areas outside the joint, and potentially cause permanent loss of range as well as intense pain. The disorder also has, on average, a 45% rate of recurrence.
The exact cause is unknown. Some doctors believe it is caused by abnormal metabolism of fat. Others think it may be caused by repetitive inflammation. Some feel that blood within the joint may cause the inflammatory change. Risk factors for PVNS developing are not yet understood. Very little research has been carried out. However, a common theme in patients is a trauma experienced to the joint prior to the onset of symptoms.
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