This is the first description of the cooccurrence of CRPS in a patient with Still's disease.
Noxious events, including minor trauma, bone fracture, or surgery of the affected limb, often determine the onset of CRPS I. Occasionally, the disease develops after other medical events such as shoulder trauma, myocardial infarction, or a lesion of the central nervous system. In the present case, the patient had a previous carpal tunnel syndrome surgery performed at her wrist. In fact, several studies have demonstrated that the surgical stimulus may produce the clinical picture of CRPS.
Regarding treatment, nonsteroidal anti-inflammatory drugs have not been demonstrated to have significant analgesic properties in CRPS. The use of opioids in CRPS has not been studied. Tricyclic antidepressants are the most well-studied medications in the context of neuropathic pain, and they have shown an analgesic effect. Glucocorticoids taken orally have clearly demonstrated efficacy in controlled trials [4
]. There is no evidence that other immune-modulating therapies, notably intravenous immunoglobulins or immunosuppressive drugs, are effective in the treatment of CRPS. Subcutaneous calcitonin only had a mild effect on spontaneous pain [5
]. However, bisphosphonates (alendronate, clodronate) induced significant improvement in pain, swelling, and movements [6
Clinical experience and two prospective studies indicate that physiotherapy is of the utmost importance in achieving the recovery of function and rehabilitation [7
Inflammation may also play a role in this unique association of Still's disease and CRPS. In fact, an increased inflammatory response is an important pathophysiological mechanism in CRPS [9
]. Indeed, some of the clinical features of CRPS, particularly in its early phase, could be explained by an inflammatory process [10
]. Consistent with this idea, corticosteroids are often successfully used to treat acute CRPS [4
]. There is increasing evidence that localized neurogenic inflammation might be involved in the generation of acute edema, vasodilatation, and increased sweating. Scintigraphic investigations using radiolabelled immunoglobulins show extensive plasma extravasation in patients with acute CRPS I [11
]. Analysis of joint fluid and synovial biopsies in CRPS patients has revealed an increase in protein concentration, synovial hypervascularity, and neutrophil infiltration [12
]. Furthermore, synovial effusion is enhanced in affected joints, as determined using MRI [13
]. In acute untreated CRPS I patients, protein extravasation elicited by strong transcutaneous electrical stimulation was only provoked on the affected extremity compared with the normal side, indicating that substance P might be involved [14
In summary, our case represents the first adult patient with Still's disease who had associated CRPS that recurred after hand surgery. Either this operation or the inflammation itself may have triggered CRPS development in this patient.