 |
|
|
 |
Development of Polymyalgia Rheumatica in Patients with Scleroderma To the Editor: Systemic sclerosis (SSc) and polymyalgia rheumatica (PMR) are uncommon but not rare rheumatic diseases. Nonetheless, we have been able to identify only 2 previous case reports of these diseases occurring in the same patient1,2. We now describe 4 patients with established SSc who were subsequently diagnosed with PMR. The various musculoskeletal symptoms that accompany SSc can make the recognition of PMR challenging in these patients3,4. In addition, the standard therapy for PMR is corticosteroids, which have been implicated in precipitating scleroderma renal crisis (SRC)5. Thus, these cases have significance for the potential diagnostic and management problems that arise when these 2 disorders occur together. Clinical profiles of our 4 patients are summarized in Table 1. All patients were female (mean age at onset of SSc 59 yrs; mean age at onset of PMR 64 yrs) and 3 of 4 patients had the diffuse cutaneous form of the disease (dcSSc). One patient previously had SRC, but had a stable creatinine of 1.8 mg/dl when PMR was diagnosed. Each patient responded appropriately to low-dose prednisone (mean initial dose 11.9 mg/day) and was successfully tapered. None experienced hypertension or renal compromise while receiving corticosteroids. Experience with the patients described here provides a framework to discuss the co-occurrence of PMR and SSc, and in particular the unique management issue related to the use of corticosteroids in SSc.
The prevalence of scleroderma has been estimated to be between 4 and 253 cases/million6. PMR/temporal arteritis occurs in patients over the age of 50 years at a prevalence of 700 cases/100,000 population6. Based on a review of the cases noted here, we suspect that PMR and SSc occur together more frequently than suggested by the paucity of articles in the literature. The 4 were identified from a cohort of about 80 patients with SSc actively followed at our institution during this period of observation. However, these data from a single center are unlikely to be a valid estimate of the incidence or prevalence of PMR in the general SSc population. Musculoskeletal symptoms in SSc occur frequently and include arthralgia, inflammatory arthritis, myopathy, and frank myositis3,4. Diffuse soft tissue swelling may occur early in the course of the disease during the edematous or inflammatory stage of SSc. This presentation can be difficult to distinguish from inflammatory arthritis or another systemic rheumatic disease. Subsequently, patients can develop a mildly inflammatory polyarthritis, which may be erosive. Patients with SSc frequently have muscle involvement, ranging from disuse atrophy to inflammatory myositis, with elevation of creatine kinase concentrations. Finally, patients may also experience musculoskeletal symptoms secondary to osteoarthritis or soft tissue rheumatism. Given the variety of musculoskeletal symptoms that can occur in SSc, recognizing PMR can be challenging. Several reports previously suggested a causal relationship between the use of corticosteroids and the precipitation of SRC5,7. SRC is defined as rapidly progressive renal failure with or without the new onset of accelerated hypertension. This complication occurs in about 10% of all patients with SSc and up to 20% of patients with dcSSc8. While SRC has mostly been reported in patients with dcSSc, in a study from the University of Pittsburgh, 3 of 60 patients with SRC were classified as having CREST syndrome (calcinosis, Raynaud's, esophageal dysmotility, sclerodactyly, telangiectasias)9. Risk factors associated with SRC include early disease, rapidly progressive skin thickening, prednisone use, and congestive heart failure7,9. Three of the 4 patients in this report had dcSSc and 2 developed PMR within 2.5 years of being diagnosed with SSc. One-year survival for patients with SRC treated with angiotensin-converting enzyme (ACE) inhibitors is 76% compared to 15% in patients in the pre-ACE-inhibitor era10. Despite significant improvements in treating this life-threatening complication, SRC continues to be an important cause of early morbidity and mortality in patients with SSc. In 1998, Steen and Medsger attempted to define the relationship between corticosteroids and the risk of SRC by reviewing their experience with a large population of patients with SSc5. That case-control study analyzed 110 patients with SRC and compared them to controls matched for age and sex. They found that the addition of high-dose corticosteroids (>= 15 mg prednisone/day) within the previous 6 months was a significant risk factor for development of SRC (OR 4.37). The addition of low-dose corticosteroids (< 15 mg prednisone daily) was not associated with a statistically significant increased risk. We conclude that PMR may develop in the setting of well established SSc and that an appropriate index of clinical suspicion be raised if a characteristic symptom complex develops with supporting laboratory data. While the prevalence of these 2 disorders occurring together is not known, we suspect that it is higher than previously reported. While there is concern regarding the use of corticosteroids in patients with SSc, standard low-dose corticosteroids were well tolerated in our 4 cases. This is consistent with the analysis by Steen and Medsger that prednisone doses < 15 mg/day were not associated with an increased risk of SRC5. However, further studies are needed to confirm these findings and we would still recommend caution in these patients.
IRENE M. ORZANO, MD, Rheumatology Fellow; EDWARD V. LALLY, MD, Director, Division of Rheumatology, Department of Medicine, Rhode Island Hospital and Brown Medical School, Associate Professor of Medicine, Brown Medical School, 2 Dudley Street, Suite 370, Providence, Rhode Island 02905, USA. REFERENCES
1. Perez-Jimenez F, Lopez-Rubio F, Canadillas F, Jimenez-Alonzo J, Jimenez-Pereperez J. Giant cell arteritis associated with 2. Hupp SL. Giant cell arteritis associated with progressive systemic sclerosis. J Neuroophthalmol 1989;9:126-30. 3. Seibold JR. Scleroderma. In: Harris Jr ED, Budd RC, Firestein GS, et al, editors. Kelley's textbook of rheumatology. 7th ed. Philadelphia: W.B. Saunders; 2004:1279-306. 4. Pope JE. Musculoskeletal involvement in scleroderma. Rheum Dis Clin North Am 2003;29:391-408.
5. Steen VD, Medsger TA Jr. Case-control study of corticosteroids and other drugs that either precipitate or protect from the 6. Lawrence RC, Helmick CG, Arnett FC, et al. Estimates of the prevalence of arthritis and selected musculoskeletal disorders in the United States 1998;41:778-99.
7. DeMarco PJ, Weisman MH, Siebold JR, et al. Predictors and 8. Steen VD. Scleroderma renal crisis. Rheum Dis Clin North Am 1996;22:861-78. 9. Steen VD, Medsger TA Jr, Osial TA Jr, Ziegler GL, Shapiro AP, Rodnan GP. Factors predicting development of renal involvement in progressive systemic sclerosis. Am J Med 1984;76:779-86. 10. Steen VD, Costantino JP, Shapiro AP, Medsger TA Jr. Outcome of renal crisis in systemic sclerosis: relation to availability of angiotensin converting enzyme inhibitors. Ann Intern Med 1990;113:352-7.
|
