A 51-year-old man was referred to our unit with a 12-month history of progressive impairment of hand function. He was unable to make a fist and had difficulty picking up small objects. He did not describe any joint pain, swelling, or morning stiffness, and there were no features to suggest an inflammatory arthropathy. He had been diagnosed with type 1 diabetes at age 7 years, complicated by diabetic nephropathy requiring a renal transplant 20 years previously (for which he was on long-term ciclosporin) and retinopathy.
On examination, his skin appeared slightly thickened. He had contractures evidenced by a positive prayer sign and was unable to flatten his hands completely. The remainder of the physical examination was unremarkable except for a functioning renal transplant; specifically, he had no evidence of synovitis or neuropathy. Laboratory investigations, including erythrocyte sedimentation rate, C-reactive protein, and rheumatoid factor, were normal. His HbA1c was 6.0%. Hand radiographs were unremarkable. He was treated with physiotherapy and wax for a presumed diagnosis of diabetic cheiroarthropathy with little improvement in his symptoms. He had a successful pancreatic transplant 2 months later, and his immunosuppression was changed to mycophenolate mofetil, tacrolimus, and a reducing course of prednisolone. His symptoms began to improve within a few weeks of surgery, and 6 weeks later he had full range of movement in both hands. His HbA1c was reduced to 5.2%. One year later, he remains asymptomatic with normal hand function.
Diabetic cheiroarthropathy is characterized by skin thickening and restriction defined as “the inability to extend the metacarpophalangeal joints fully” (1) and is thought to be caused by collagen abnormalities and increased glycation of connective tissue. Studies suggest that cheiroarthropathy is associated with type 1 diabetes (2), duration of diabetes (3,4), and secondary complications (2,4). Treatment is often unsatisfactory, involving corticosteroid injection or surgery in severe cases. It is suggested that improved glycemic control may improve symptoms, although it is not usually associated with complete resolution.
Pancreatic transplantation is currently the only therapy for type 1 diabetes that re-establishes endogenous insulin secretion, rendering the recipient euglycemic. Follow-up studies of pancreatic transplant patients suggest that complications, including retinopathy, nephropathy, and neuropathy, are stabilized. Several studies report reversal of nephropathy (5,6), although it is suggested this can only be expected after a long observation period (6).
This is the first case we are aware of in which cheiroarthropathy has been noted to resolve posttransplantation and highlights two potential mechanisms of cheiroarthropathy. Firstly, the early improvement on corticosteroids suggests an inflammatory component, which other authors have postulated (7), although the sustained improvement suggests that this is not simply a steroid effect but may reflect other factors such as a change in immunosuppression. Secondly, an alternative hypothesis suggests that the improvement in glycemic control may lead to resolution of symptoms, although the speed of improvement makes this less likely.
In conclusion, we observed the resolution of diabetic cheiroarthropathy after successful pancreatic transplant, which raises interesting potential mechanisms of cheiroarthropathy.
