What is Psoriatic Arthritis Mutilans: Symptoms, Causes, Treatment, Risk Factors, Diagnosis

What is Psoriatic Arthritis Mutilans?

Psoriasis is a common autoimmune disease, and it is estimated that nearly two to three percent of the world’s population is affected by psoriasis.(1,2) It is expected that almost 85 percent of people with psoriasis will go on to develop psoriatic arthritis.(3) Psoriatic arthritis is an autoimmune disease that is closely related to psoriasis. People with psoriatic arthritis experience joint pain, stiffness, and swelling, along with the skin-related symptoms of psoriasis as well.(4,5)

Psoriatic arthritis mutilans is a rare and severe form of psoriatic arthritis.(6) Psoriatic arthritis mutilans is commonly observed in people who already have psoriatic arthritis, but it can also develop in people with rheumatoid arthritis.(7) It is expected that about five percent of people with psoriatic arthritis develop this rare form of arthritis. Arthritis mutilans causes bone and tissue loss, known as osteolysis. This causes changes to the shape of the toes or fingers and can impair a person’s movement over time. This condition also causes lower back and neck pain.

Psoriatic arthritis mutilans is also referred to as ‘telescopic finger’ or ‘opera-glass hand.’ This severe form of arthritis tends to usually affect the hands, but it may also impact the fingers, feet, and wrists.(8)

In many cases, psoriatic arthritis mutilans can cause permanent damage to the feet, hands, toes, and fingers. However, studies have found that getting an early diagnosis and starting treatment early can prevent the progression of joint damage in psoriatic arthritis mutilans.(9)

Symptoms of Psoriatic Arthritis Mutilans

As psoriatic arthritis mutilans is a form of inflammatory arthritis, anyone who develops this condition will experience the symptoms of arthritis. This includes the common arthritis symptoms like stiff and painful joints and a reduced range of motion as the condition progresses. Psoriatic mutilans develops when psoriatic arthritis becomes very severe, and the inflammation caused by this condition starts to attack the bone tissue. This starts to break down the bone tissue over a period of time and the body then also starts to reabsorb the bone.(10)

When psoriatic arthritis develops into psoriatic arthritis mutilans, they may experience symptoms like:

  • Toes or fingers start to shorten and bunch together, a condition known as telescoping.(11)
  • It becomes challenging or almost impossible to move the joints in the feet and hands.
  • The loose skin starts bunching up over the shortened toes and fingers.
  • Bones start to fuse, a condition known as ankylosis.(12)

Other abnormalities may also be noticed with the anatomy of the fingers and toes. People with psoriatic arthritis mutilans also experience the symptoms of psoriatic arthritis and sometimes psoriasis as well. These can include:

  • Fatigue
  • Joint stiffness and swelling.
  • Joint pain or throbbing pain throughout the body.
  • Red patches of skin with raised, silvery scales or silvery patches of skin.
  • Pain, tenderness, and swelling of the tendons.
  • Uveitis, a condition that causes painful red eyes along with blurred vision.

Over a period of time, people who have psoriatic arthritis mutilans experience bone damage, and the bone in the affected joint may slowly disappear altogether. This makes it almost impossible to bend or straighten that affected joint.

The affected joints may shorten as well, causing loose skin to develop in these affected parts. This loose skin retracts, becoming mobile. This is also why one of the names of this condition is telescopic fingers.

What are the Causes of Psoriatic Arthritis Mutilans?

There are five types of psoriatic arthritis, of which psoriatic arthritis mutilans is the most severe form of psoriatic arthritis.(13) In order to understand what causes psoriatic arthritis mutilans, it is necessary to first understand how psoriatic arthritis develops.

Psoriatic arthritis usually develops in people who already have psoriasis. Psoriasis is an autoimmune disease in which your immune system starts to mistakenly attack the healthy cells. This causes inflammation throughout the body, including your joints. It also causes joint stiffness and pain. This inflammation is the leading cause of psoriatic arthritis.

In the long run, the continued inflammation of your joints can lead to permanent damage. Some bones, especially the ones that are connected to frequently used joints like the hands and feet, will begin to erode. This severe stage of psoriatic arthritis is known as psoriatic arthritis mutilans. The acute inflammation that affects the bones and joints, over time, causes the body to absorb the bone tissue. This results in bone loss and deformities of the hand and feet.(14)

However, the exact cause of the faulty and aggressive immune response in autoimmune diseases like psoriatic arthritis is not yet clear. However, it is believed to have a genetic component as studies have found several genes that are known to increase the risk of developing psoriasis and psoriatic arthritis.(15,16) It is estimated that around 40% of all people with psoriatic arthritis have a family member who has either psoriasis or arthritis.(17)

Nevertheless, genetics is not the only cause behind psoriatic arthritis mutilans. Researchers believe that many environmental factors, including infections or injuries, can also impact a person’s risk of developing such types of psoriatic disorders.

What are the Risk Factors of Psoriatic Arthritic Mutilans?

Psoriatic arthritis mutilans is a very rare condition. However, there is very little evidence on how to predict who will develop this condition. What is known is that people who have psoriasis but not psoriatic arthritis will not develop psoriatic arthritis mutilans. Current research into the various risk factors for psoriatic arthritis mutilans remains inconclusive. Diagnosis of psoriasis at a young age and childhood obesity are considered to be risk factors. However, so far, the only proven risk factor of developing psoriatic arthritis mutilans is a genetic link or having a family history of this condition.(18)

However, researchers of a recent case study stated that with the advancement and effectiveness of treatments today for psoriatic arthritis, the incidence of psoriatic arthritis mutilans has become extremely rare.(19) Many of the reported cases that have been documented in medical literature show that psoriatic arthritis mutilans usually develops when a person with psoriatic arthritis does not receive treatment or stops treatment in between.(20)

Therefore, the risk of a person getting psoriatic mutilans even if they have psoriatic arthritis is relatively low, especially if they continue taking their medications to control the inflammation caused by this autoimmune condition.

Diagnosing Psoriatic Arthritis Mutilans

In many cases, people who have been diagnosed with psoriatic arthritis do not know they have psoriasis.(21) In fact, in 85 percent of all cases of psoriatic arthritis, the symptoms of psoriasis become apparent before the symptoms of arthritis become apparent.(22)

For diagnosing psoriatic arthritis mutilans, your doctor will first run tests to diagnose if you have arthritis. This is done by checking your joints for tenderness and signs of swelling. You will also have to undergo diagnostic tests. Your doctor is likely to order tests that check for the presence of specific arthritis-related antibodies and inflammation markets. You will also need to undergo an X-ray and other imaging tests to assess the actual damage to your joints.

Once you have been diagnosed with arthritis, your doctor will order a blood test to determine what type of arthritis you have. For example, if the blood test results reveal the presence of cyclic citrullinated peptide (CCP) and rheumatoid factor (RF) antibodies in the blood, you are likely to have rheumatoid arthritis.(23)

Even though currently there is no lab biomarker for psoriatic arthritis mutilans or psoriatic arthritis. In this case, psoriatic arthritis mutilans is therefore diagnosed by checking the severity of damage caused to the bones and whether there is any loss of bone tissue. Except for psoriatic arthritis mutilans, there are very few medical conditions that can cause such severe bone loss and joint degradation.

What is the Treatment for Psoriatic Arthritis Mutilans?

The earlier psoriatic arthritis mutilans is diagnosed, the more likely it is that the treatment will help stop the progression of the disease. This is a progressive disease, meaning it gets worse as time progresses. Since there is no cure for psoriatic arthritis, the primary goal of treating psoriatic arthritic mutilans revolves around managing the symptoms and helping people improve their quality of life with an autoimmune disease.

There are various treatments for this condition, and most of them include disease-modifying anti-rheumatic drugs (DMARD) and methotrexate (brand name Trexall), which is an anti-TNF inhibitor. Many treatment plans use either one of these options or both.(24)

Methotrexate helps relieve the symptoms of arthritis, but it is yet unclear whether this medication can slow down bone loss.

Studies have shown that medication known as anti-TNF inhibitors can help stop the symptoms of psoriatic arthritis from further aggravating or getting worse.(25) These anti-TNF inhibitors can alter the body’s anti-inflammatory responses. This helps suppress the inflammation and avoids the joints feeling painful or stiff. These medications may also help boost joint function to some level. A 2011 study found that the drug Enbrel (etanercept) helps restore some function to the affected joints.(26)

Conclusion

Psoriatic arthritis mutilans can cause permanent disability if left untreated. However, over the years, due to advancements in medications and treatment options, a diagnosis of psoriatic arthritis mutilans is no longer as drastic as it used to be earlier. It is also essential to keep in mind that the outlook for the diseases improves significantly when psoriatic arthritis is detected and treated at an early stage. Early treatment also helps prevent bone loss and stops psoriatic arthritis from progressing into psoriatic arthritis mutilans.

References:

  1. Mohd Affandi, A., Khan, I. and Ngah Saaya, N., 2018. Epidemiology and clinical features of adult patients with psoriasis in Malaysia: 10-year review from the Malaysian Psoriasis Registry (2007–2016). Dermatology research and practice, 2018.
  2. Parisi, R., Symmons, D.P., Griffiths, C.E. and Ashcroft, D.M., 2013. Global epidemiology of psoriasis: a systematic review of incidence and prevalence. Journal of Investigative Dermatology, 133(2), pp.377-385.
  3. Vaengebjerg, S., Skov, L., Egeberg, A. and Loft, N.D., 2020. Prevalence, incidence, and risk of cancer in patients with psoriasis and psoriatic arthritis: a systematic review and meta-analysis. JAMA dermatology, 156(4), pp.421-429.
  4. Moll, J.M.H. and Wright, V., 1973, January. Psoriatic arthritis. In Seminars in arthritis and rheumatism (Vol. 3, No. 1, pp. 55-78). WB Saunders.
  5. Ritchlin, C.T., Colbert, R.A. and Gladman, D.D., 2017. Psoriatic arthritis. New England Journal of Medicine, 376(10), pp.957-970.
  6. Haddad, A. and Chandran, V., 2013. Arthritis mutilans. Current rheumatology reports, 15(4), p.321.
  7. Helliwell, P.S., Porter, G. and Taylor, W.J., 2007. Polyarticular psoriatic arthritis is more like oligoarticular psoriatic arthritis, than rheumatoid arthritis. Annals of the rheumatic diseases, 66(1), pp.113-117.
  8. Haddad, A. and Chandran, V., 2013. Arthritis mutilans. Current rheumatology reports, 15(4), p.321.
  9. Mochizuki, T., Ikari, K. and Okazaki, K., 2018. Delayed Diagnosis of Psoriatic Arthritis Mutilans due to Arthritis Prior to Skin Lesion. Case Reports in Rheumatology, 2018.
  10. Jadon, D.R., Shaddick, G., Tillett, W., Korendowych, E., Robinson, G., Waldron, N., Cavill, C. and McHugh, N.J., 2015. Psoriatic arthritis mutilans: characteristics and natural radiographic history. The Journal of rheumatology, 42(7), pp.1169-1176.
  11. Oliveira, M., Marinho, A., Gomes, V., Meireles, M. and Patinha, F., 2016. Telescopic fingers: The severe form of arthritis mutilans. Indian Journal of Rheumatology, 11(2), pp.114-115.
  12. Haddad, A. and Chandran, V., 2013. Arthritis mutilans. Current rheumatology reports, 15(4), p.321.
  13. Arthritis.org. 2020. [online] Available at: <http://www.arthritis.org/about-arthritis/types/psoriatic-arthritis/what-is-psoriatic-arthritis.php> [Accessed 27 November 2020].
  14. Dhir, V. and Aggarwal, A., 2013. Psoriatic arthritis: a critical review. Clinical reviews in allergy & immunology, 44(2), pp.141-148.
  15. Haroon, M., Winchester, R., Giles, J.T., Heffernan, E. and FitzGerald, O., 2017. Clinical and genetic associations of radiographic sacroiliitis and its different patterns in psoriatic arthritis. Clin Exp Rheumatol, 35(2), pp.270-276.
  16. Nograles, K.E., Brasington, R.D. and Bowcock, A.M., 2009. New insights into the pathogenesis and genetics of psoriatic arthritis. Nature clinical practice Rheumatology, 5(2), pp.83-91.
  17. Rheumatology.org. 2020. Diseases And Conditions Psoriatic Arthritis. [online] Available at: <https://www.rheumatology.org/I-Am-A/Patient-Caregiver/Diseases-Conditions/Psoriatic-Arthritis> [Accessed 27 November 2020].
  18. Ogdie, A. and Gelfand, J.M., 2010. Identification of risk factors for psoriatic arthritis: scientific opportunity meets clinical need. Archives of dermatology, 146(7), pp.785-788.
    Ferreira, M.B., Sá, N., Rocha, S.M. and Marinho, A., 2013. Opera glass hands: the phenotype of arthritis mutilans. BMJ case reports, 2013.
  19. Managedhealthcareconnect.com. 2020. [online] Available at: <https://www.managedhealthcareconnect.com/articles/consequences-neglecting-rheumatoid-arthritis-treatment-case-arthritis-mutilans-older-woman> [Accessed 27 November 2020].
  20. Rheumatology.org. 2020. Diseases And Conditions Psoriatic Arthritis. [online] Available at: <http://www.rheumatology.org/I-Am-A/Patient-Caregiver/Diseases-Conditions/Psoriatic-Arthritis> [Accessed 27 November 2020].
  21. Psoriasis.org. 2020. Are You At Risk For Psoriatic Arthritis?. [online] Available at: <https://www.psoriasis.org/psoriatic-arthritis/diagnosis> [Accessed 27 November 2020].
  22. Schellekens, G.A., Visser, H., De Jong, B.A., Van Den Hoogen, F.H., Hazes, J.M., Breedveld, F.C. and Van Venrooij, W.J., 2000. The diagnostic properties of rheumatoid arthritis antibodies recognizing a cyclic citrullinated peptide. Arthritis & Rheumatism: Official Journal of the American College of Rheumatology, 43(1), pp.155-163.
  23. Nash, P. and Clegg, D.O., 2005. Psoriatic arthritis therapy: NSAIDs and traditional DMARDs. Annals of the rheumatic diseases, 64(suppl 2), pp.ii74-ii77.
  24. Benedict, W.F., Baker, M.S., Haroun, L., Choi, E. and Ames, B.N., 1977. Mutagenicity of cancer chemotherapeutic agents in the Salmonella/microsome test. Cancer research, 37(7 Part 1), pp.2209-2213.
  25. Eder, L., Chandran, V. and Gladman, D.D., 2011. Repair of radiographic joint damage following treatment with etanercept in psoriatic arthritis is demonstrable by 3 radiographic methods. The Journal of rheumatology, 38(6), pp.1066-1070.

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