Pigmented Villonodular Synovitis (PVNS)

Belle is one of our interns at the Hilliard office this past Summer 2019. She was fascinated by Pigmented Villonodular Synovitis or PVNS for short and was first introduced to it on her first day with one of her first patients as an intern. This was something that has stuck with her since she started here and something that she found really interesting. Read below to learn what she discovered!

What is PVNS?

PVNS is a proliferative condition (fast growing) that affects primarily the synovium (the thick flexible membrane forming a sac) of the joints; however, it can affect the other tissue around the joints like the bursae or the tendon sheaths. In this condition, the synovium creates extra fluid which causes inflammation and the tissue thickens.

During this process, benign tumors grow within the soft tissues of the joints. When left untreated, it can cause destruction of the joint and the surrounding tissue. PVNS usually only involves one joint, primarily the larger joints. 80% of individuals with PVNS experience this condition in their knees; however, it can be found in the hips, ankles, elbows, shoulders, etc.

Types of PVNS

There are two types of PVNS. The first is characterized as localized. This is pretty self-explanatory as this type of PVNS is localized to one area of the joint. It is usually intra-articular and is usually found in smaller joints and is easier to treat because the tumors are smaller and in one specific area. 

The second type is called diffuse PVNS. Diffuse PVNS is the most common type of PVNS that is found in the larger joints. This is when the tumors are located around the entire joint and can be extra-articular which can affect the bursae or the tendons surrounding the joint. It is more destructive to the tissue because the larger tumors can infiltrate the tissue around the joint making treatment more difficult.

Prevalence

PVNS is classified as a rare condition with about 2 patients per million each year. Middle aged adults are primarily the patient population with the median age being 30 yo, but there are some pediatric cases. If children develop this condition early, it is most likely more progressive and polyarticular meaning that it can be found in more than one joint.

Cause

The cause of PVNS is relatively unknown. There are some theories, but most of them do not have sufficient evidence showing causation. Many believe genetics may play a part or history previous trauma, but researchers have shown a connection with the colony stimulating factor 1 or CSF1 and what is called the landscape effect.

Signs & Symptoms

Pain, inflammation, accumulation of fluid, stiffness or locking of the joint, instability, and tenderness. 

Again, if left untreated it can lead to serious damage to the joint and the soft tissue.

Treatment Options

When it comes to treatment, surgery is the gold standard. There can be a synovectomy to remove the synovium. Most of the time, resection of the tumors is the surgical procedure most patients experience.

Because joint degeneration can happen in severe cases joint replacements have been used; however, joint replacements do not treat the PVNS itself, but the joint deterioration. Surgery is usually a temporary fix because 50% of cases experience recurrence of tumors. Because the most likely origin is genetic expression, the tumors usually grow back.

Radiation therapy is also an intervention, but is usually used in conjunction with surgery. Pharmaceuticals have also developed that inhibit CSF1 receptor activation which would in turn, help to decrease the growth of tumors.

But what about physical therapy?

Physical Therapy and PVNS

There is very little research connection PT and treatment for PVNS. PT could possibly be used in all different situations like pre-op, post-op, or management of the condition.

Through my research, I found a case study conducted by a PT student from St. Catherine University. The patient was a 48 yo female who had diffuse PVNS in her left knee. She had multiple surgeries in conjunction with radiation. She experienced pain with walking, sitting and squatting, and doing basic home and at work activities. She was seen post-op 2x per week for 14 weeks. The therapy she received consisted of strengthening exercises, stretching, joint mobilizations, and addressing gait abnormalities.

Physical Therapy Case Study

After this care, the woman experience less pain, increased her knee flexion by 45%, increased her knee extension by 70%, increased her strength on both legs, and her gait abnormalities were only present during fatigue. 

In this case, PT was very helpful in treating PVNS post-op. It allowed the patient to increase her functional mobility, strength, range of motion, and feel less pain.

Conclusion

Based on what I have experienced this summer, I think that this could also be a very good intervention for pre-op patients to increase the muscles around the joint prior to surgery to help with the recovery process post-op. PT could also allow for better management of PVNS, so individuals with the condition can continue to make sure they work on those affected joints. However, like all medical care, it is patient based and on a case by case basis. Physical therapy can help these individuals before surgery, after surgery, or throughout their life to manage their condition and decrease the issues with this painful degenerative condition.

Do you or someone you know have PVNS? Or do you have knee pain? Give us a call at 614-850-0500 to schedule today!

References:

  1. Oehler, S., Fassebender, H.G., Neureiter, D., Meyer-Scholten, C., Kirchner, T., Aigner, T. (2000). Cell populations involved in pigmented villonodular synovitis of the knee. Retrieved from https://www.ncbi.nlm.nih.gov/pubmed/10685815 
  2. Pigmented villonodular synovitis. Retrieved August 8, 2019 from https://rarediseases.info.nih.gov/diseases/7396/pigmented-villonodular-synovitis
  3. Pigmented Villonodular Synovitis. Retrieved August 8, 2019 from https://rarediseases.org/physician-guide/pigmented-villonodular-synovitis
  4. Pigmented Villonodular Synovitis. Retrieved August 9, 2019 from https://orthoinfo.aaos.org/en/diseases–conditions/pigmented-villonodular-synovitis
  5. Schutte, Christa. (2015). Physical Therapy Management of a Patient with Diffuse Pigmented Villonodular Synovitis: A Case Report. Retrieved from Sophia, the St. Catherine University repository website: https://sophia.stkate.edu/cgi/viewcontent.cgi?article=1044&context=dpt_papers
  6. Stephan, S. R., Shallop, B., Lackman, R., Kim, T. W., Mulchahey, M. K. (2016). Pigmented Villonodular Synovitis: A Comprehensive Review and Proposed Treatment Algorithm. doi: 10.2106/JBJS.RVW.15.00086. 
  7. Tyler, W. K., Vidal, A. F., Williams, R. J., Healey J. H. (2006). Pigmented villonodular synovitis. doi: 14(6): 376-85UniProt ConsortiumEuropean Bioinformatics InstituteProtein Information ResourceSIB Swiss Institute of Bioinformatics. (2019, July 31). Macrophage colony-stimulating factor 1. Retrieved from https://www.uniprot.org/uniprot/P09603
No Comments

Sorry, the comment form is closed at this time.