Diabetic Cheiroarthropathy: The Musculoskeletal Complication Clinicians May Be Overlooking

by Michelle LaPlante 

With more intensive insulin therapy regimens, people with diabetes are living longer and have better control of the disease and its complications. Although severe complications such as blindness and kidney disease receive most of the attention, individuals with longstanding diabetes (approximately 30 years) may also need treatment for complications that are not life threatening but are still debilitating. One of these complications, cheiroarthropathy, is a common syndrome involving the tendons and joints. 

Consultant360 spoke with David M. Nathan, MD, Director of the Clinical Research Center and the Diabetes Center at Massachusetts General Hospital, to learn more about this little known and underappreciated syndrome that comprises conditions such as carpal tunnel syndrome, adhesive capsulitis, flexor tenosynovitis (commonly referred to as trigger finger), and Dupuytren contracture. Dr. Nathan is also a Professor of Medicine at Harvard Medical School, and he has been involved with the Diabetes Control and Complications Trial (DCCT)¹ and the long-term follow up of that study² during his 40-year career as a researcher of diabetic complications, glycemic control, and the hemoglobin A1c (HbA1c) assay.

Could you tell me more about cheiroarthropathy and why so few clinicians seem to be aware of it?

Cheiroarthropathy is an old Greek term that loosely translated means musculoskeletal disorders and, specifically, limited joint mobility. Cheiroarthropathy was identified and characterized in people with type 1 diabetes more than 40 years ago.³ However, it has been a generally underappreciated complication because other complications are more dramatic and have a major impact on people’s lives and lifespans.

In recent years, a better understanding of the intimate relationship between diabetes complications and our ability to better control diabetes has led to a reduction in severe complications such as heart disease, eye disease leading to blindness, kidney disease leading to kidney failure, and the need for transplantations and amputations.² We are fortunate that more people with diabetes, especially those with type 1 diabetes, are living longer lives as a result of these dramatic reductions in complications.

The story of diabetes care is a good news/bad news scenario though. The good news is that patients with type 1 diabetes are now living much closer to a normal life span. This is a result of  the reduction in these major complications.

The bad news is that clinicians are starting to see more and more minor complications, such as cheiroarthropathy, that may be debilitating but are not life threatening. Patients may present with stiffening and decreased flexibility of the connective tissues of shoulder joints, fingers, knees, and hips (although hips are not as well recognized), and increased thickening of structures in the wrist.^4,5

These complaints are categorized under the umbrella term of cheiroarthropathy, which means that connective tissues surrounding the joints have become thicker and less flexible over time. This leads to conditions that can have a negative impact on quality of life, including trigger fingers, carpal tunnel syndrome, and adhesive capsulitis of the shoulder. For example, although stiff fingers are not life threatening, stiffness may interfere with daily activities such as typing on keyboards or performing other office work. Adhesive capsulitis of the shoulder leads to limitations in range of motion and may prevent people from reaching above their heads or women from fastening and unfastening their bras.

I have mostly discussed cheiroarthropathy in people with type 1 diabetes. Although type 1 diabetes is the less common form of diabetes, it is the form we have studied longest and know best. It is also where most of these cheiroarthropies, or musculoskeletal conditions, have been diagnosed. These complications also occur in type 2 diabetes, but have not been studied as carefully as in people with type 1 diabetes.
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In addition to the data you mentioned, is cheiroarthropathy usually discussed in people with type 1 diabetes because they have often have had diabetes longer than those diagnosed with type 2 diabetes, and thus have had more time to develop complications?

What is probably happening is that the stiffening of the connective tissue is occurring after decades of exposure to higher blood sugar levels.⁵ Patients with type 1 diabetes develop diabetes earlier in their lives, which means that they are more vulnerable to being exposed to elevated blood sugar levels for a longer period of time.

We think that the improvement in glucose control that has led to the reduction in kidney, eye, and nerve damage should also ultimately benefit this particular form of diabetic complications. It is worth pointing out that individuals do not have to have diabetes to develop a frozen shoulder, carpal tunnel syndrome, or Dupuytren contractures. However, diabetes does dramatically increase the risk of these conditions.

What are some strategies that you suggest for coordinating and communicating between the specialists who treat these conditions?

Cheiroarthropathy symptoms often manifest in the hands, shoulders, elbows, and wrists, so patients are often referred to a rheumatologist or orthopedist for a steroid injection or surgery. Internal medicine specialists, and diabetes specialists in particular, may see their patients present with stiffness in the hands or shoulder, triggering or locking of their fingers, or carpal tunnel syndrome associated with the thickening of soft tissue structures in the wrist. Thus, they also need to be equipped to better recognize the symptoms of cheiroarthropathy so that they can refer the patient to a specialist for treatment.

Because the symptoms of cheiroarthropathy often mimic those of arthritis, is cheiroarthropathy often misdiagnosed by clinicians who are unfamiliar with this musculoskeletal complication?

In our office we have been paying close attention to cheiroarthropathy for at least the last 20 years, so our staff is used to dealing with the diagnosis, and we do not often misdiagnose. However, the diagnosis can certainly be difficult, because both cheiroarthropathy and arthritis are seen with aging, and individuals can have elements of both conditions.

Cheiroarthropathy is really different from arthritis though. It is a periarthritis. I imagine that there may be some confusion among non-specialists regarding this distinction, especially since both cheiroarthropathy and arthritis can co-exist in the same person.

Are steroids a treatment option for cheiroarthropathy? (Continued on the next page)

Steroid injections do work, although they may not work in every individual, and their beneficial effects may be transient. There is an inflammatory component around cheiroarthropathy, which is why the steroid injections may work for some individuals and may functionally relax the collagen a little bit. Some patients with a trigger finger or adhesive capsulitis may respond to their first local steroid injection and not need another treatment.

However, it is important to note that trigger finger in patients with type 1 diabetes may affect numerous fingers. If the beneficial effects of a first steroid injection stop, a second injection may be necessary The second injection may work, but for an even shorter period of time, and then patients may eventually need surgical release of the tendon. The bottom line is that we would always like to avoid surgery if we can, but we also need to recognize when the current therapy is not effective and when surgery is needed. Recognizing the appropriateness of surgery is important. Fortunately, the tendon release is a minor procedure, usually performed with local anesthesia, with full recovery over a week or so.

What is different in a person with diabetes that causes these physiologic changes in the tendons?

Anyone can have thickening of these tendons. In fact, there are some familial situations in which tendons are less flexible and thicker. However, in people with diabetes, elevated blood sugar levels bind to a wide variety of proteins, so-called glycation. The binding of glucose to the red cell protein hemoglobin is the basis of the HbA1c assay.

The same phenomenon occurs with collagen, the major component of our connective tissues. Over many years, the glucose binds to the collagen, and changes the physical characteristics of the collagen―it makes the collagen stiffer.

We know this mostly from animal studies. The collagen in people with type 1 diabetes, if exposed to even slightly higher glucose over many years, becomes thicker, and tendons and the structures through which they slide, thicken and become less flexible. In the palm of the hand, the tendons that usually slide through connective tissue “tunnels” or “pulleys” become stuck because the structures are thicker, thus resulting in triggering.

The same process occurs around other joints, explaining why people with diabetes experience stiffening in their hands and shoulders, and are at increased risk for carpal tunnel syndrome. However, in the case of carpal tunnel syndrome, the connective tissue tunnel is squeezing a nerve, not a tendon. When collagen thickens in the shoulder, the diagnosis is adhesive capsulitis.

What are the risk factors for cheiroarthropathy besides diabetes and higher A1cs?

Those are the major risk factors, but there is also a genetic component, which we do not understand well at this time. Some people will have a history of nondiabetic relatives who have had numerous episodes of these cheiroarthropathies and who have required surgery. However, the primary risk factors are the increased level of glucose and the duration of the exposure—in other words, the number of years an individual has had diabetes.

What is the best method for determining if a patient has cheiroarthropathy?

Start by examining the patient to see if he or she is experiencing decreased mobility of the hands, which is the area that we worry about the most, since we use our hands in all phases of our daily life. Patients may also present with shoulder pain and report discomfort when trying to reach for an object on a shelf or when putting their hand behind their head.

We rely on our fingers for so many activities in our daily life, but it is not clear if being more active inhibits the development of cheiroarthropathy or exacerbates it. We are seeing more patients with cheiroarthropathy in their hands, but we are not sure whether this increase in cases is because we depend on our fingers more than in the past, or because increased use of keyboards and our hands in general means that we simply notice these disabilities more than in the past.

Clinicians examining a patient with type 1 or type 2 diabetes should check flexibility of the hands in the physical examination. An easy test to determine hand flexibility is the prayer sign test,⁶ which has the patient placing his or her palms together, fingers right against the other, and looking to see if there is any air space between the fingers. People who are starting to see a thickening around their knuckles will have what is called a positive prayer sign, which means that when they try to put their fingers together, they can’t and you can look right through the fingers. It is an early indication that the connective tissue is starting to become stiffer. People with a positive prayer sign may have decreased flexibility of their fingers and less strength in their hands.

Another test a clinician can give to a patient is the grip test: ask the patient to squeeze your fingers as hard as they can, and if they have a weak grasp, then that is a sign of a problem. Ask the patient about finger triggering, carpal tunnel syndrome, and shoulder pain and flexibility, and consider following up with a referral to a hand specialist or an orthopedist if they answer in the affirmative. Note that these symptoms can be painless, such as with flexor tenosynovitis and carpal tunnel syndrome, but the shoulders are often painful. Treatment is aimed at relieving discomfort and restoring functional mobility.

A third test to check for symptoms of cheiroarthropathy is to have the patient put his hand in front of him, parallel to the floor, and then have him try to bring the arm directly over his head to see if he can raise it parallel with the ear. Testing internal and external rotation at the shoulder is also important.

How do you know the difference between arthritis and cheiroarthritis?

Recognizing the difference comes from experience. There are differences between arthritis and cheiroarthritis, especially in the type 1 population in which patients often develop periarthritis symptoms before they are old enough to develop real arthritis. Remember, people can develop wear-and-tear arthritis on joints that bear weight, such as ankles, knees, and hips, and in fingers.

Although fingers do not bear weight, they must remain flexible to be functional. Even without weight bearing, it is still possible to develop arthritis in finger joints, but this usually occurs at an advanced age and is more common in people who engage in physical labor with their hands. Thus, there is no question that individuals can develop arthritis in the hands, but cheiroarthropathy is a bit different. Clinicians need to understand it to diagnose it properly.

What is the relationship between cheiroarthropathy and microvascular complications?

They do all go together, but we are seeing more and more people who survive with diabetes for many decades (and who have escaped the eye, kidney, and nerve diseases) go on to develop musculoskeletal complications. The various complications are connected, and people with out-of-control diabetes with higher A1cs are more likely to develop cheiroarthropathy as well as other complications. However,  the connective tissues appear to be very sensitive, even to modest elevations in glucose levels.⁷

So you might see cheiroarthropathy in patients who have no other complications?

That is often who we are seeing it in. People who have been in pretty good glucose control, and who have avoided major complications, may still have minor complications such as cheiroarthropathy. These complications need to be detected and addressed.

References

1. The Diabetes Control and Complications Trial Research Group. The effect of intensive treatment of diabetes on the development and progression of long-term complications in insulin-dependent diabetes mellitus. N Engl J Med. 1993;329(14):977-986.

2. Nathan DM. The diabetes control and complications trial/epidemiology of diabetes interventions and complications study at 30 Years: overview. Diabetes Care. 2014;37(1):9-16.

3. Grgic A, Rosenbloom AL, Weber FT, Giordano B, Malone JI, Shuster JJ. Joint contracture—common manifestation of childhood diabetes mellitus. J Pediatr. 1976;88(4 Pt 1):584-588.

4. Rosenbloom AL. Limited joint mobility in childhood diabetes: discovery, description, and decline. J Clin Endocrinol Metab. 2013;98(2):466-473.

5. Larkin ME, Barnie A, Braffett BH, et al. Musculoskeletal complications in type 1 diabetes. Diabetes Care. 2014;37(7):1863-1869.

6. Raina S, Jaryal A, Sonnatakke T. Prayer sign. Indian Dermatol Online J. 2013;4(3):259. doi:10.4103/2229-5178.115545.

7. Gerrits EG, Landman GW, Nijenhuis-Rosien L, Bilo HJ. Limited joint mobility syndrome in diabetes mellitus: a minireview. World J Diabetes. 2015;6(9):1108-1112.