Correspondence
Case 26-2001: Scleroderma Renal Crisis and Polymyositis
N Engl J Med 2002; 346:1916-1918June 13, 2002
- Article
To the Editor:
We believe that an important myositis-associated autoantibody, which could be of interest in the differential diagnosis, was overlooked by Drs. Korn and Mauiyyedi in their discussion of Case 26-2001 (Aug. 23 issue).1 Anti–PM-Scl, formerly known as anti-PM1, reacts against an antigen of at least 11 subunits, although the major antigen is the 100-kD protein. Almost all anti–PM-Scl autoantibodies react with it. This autoantibody is found in scleromyositis or the overlap syndrome of systemic sclerosis and polymyositis, the clinical diagnosis made by the physicians attending the patient. Even though anti–PM-Scl antibody can be found in patients with polymyositis and systemic sclerosis alone, it is usually found in patients with scleromyositis. Although the antibody is rare (present in 4 percent in a series of 617 patients with various connective-tissue diseases, in 8 percent in a series of 168 patients with myositis, and in 12 percent in our series of 62 Spanish patients with inflammatory idiopathic myopathies), it has been clearly related to the overlap syndrome of myositis and systemic sclerosis. Anti–PM-Scl is best detected by protein and RNA immunoprecipitation and appears to delineate a subgroup of patients with clinical characteristics of myositis and systemic sclerosis, which could have a course as discussed in the case.
1 ReferencesAlbert Selva O'Callaghan, M.D., Ph.D.
Moises Labrador Horrillo, M.D., Ph.D.
Miquel Vilardell Tarrés, M.D., Ph.D.
Vall d'Hebron General Hospital, 08035 Barcelona, Spain
aselva@hg.vhebron. es 1
Case Records of the Massachusetts General Hospital (Case 26-2001). N Engl J Med 2001;345:596-605
Full Text | Web of Science | Medline
To the Editor:
The patient presented in Case 26-2001 was initially given a diagnosis of polymyositis. A regimen of alendronate was begun at the same time that high-dose glucocorticoid therapy was initiated. In this regard, her physicians were following recently published guidelines on the prevention of osteoporosis.1 However, this patient also had a history of “difficulty swallowing solid foods,” and it is noted that her dysphagia subsequently worsened. Alendronate is contraindicated in patients with abnormalities of the esophagus that delay esophageal emptying2 because esophageal ulceration is a potential complication of therapy.3 In this patient, who was ultimately given a diagnosis of the overlap syndrome of scleroderma and polymyositis, oral bisphosphonate therapy may have contributed to rapid worsening of her dysphagia. Intravenous therapy with pamidronate may be a safer option for prevention or treatment of osteoporosis in patients with scleroderma or polymyositis.4
4 ReferencesLee S. Shapiro, M.D.
Center for Rheumatology, Albany, NY 12206
lshapiro@joint-docs.com 1
American College of Rheumatology Ad Hoc Committee on Glucocorticoid-Induced Osteoporosis
CrossRef | Web of Science | Medline2
Fosamax: prescribing information. Whitehouse Station, N.J.: Merck, 2001. (Accessed May 10, 2002, at http://www.fosamax.com.)
3
Colina RE ,Smith M ,Kikendall JW ,Wong RK . A new probable increasing cause of esophageal ulceration: alendronate. Am J Gastroenterol 1997;92:704-706
Web of Science | Medline4
Watts NB . Treatment of osteoporosis with bisphosphonates. Rheum Dis Clin North Am 2001;27:197-214
CrossRef | Web of Science | Medline
To the Editor:
A muscle biopsy from the patient in Case Record 26-2001 showed “myositis,” yet the result of a later magnetic resonance imaging (MRI) examination “was consistent with the presence of polymyositis.”
Why MRI was performed is unclear. Such an investigation, at a minimum, adds extra cost to the care of the patient, yet extra investigations can have other consequences.1,2 I cannot see what the MRI added to the care of this patient, since the results were only confirmatory.
2 ReferencesDominic Aldington, M.B., B.S.
Royal Hospital Haslar, Gosport PO12 2AA, United Kingdom
daldington@mac.com 1
Schein OD ,Katz J ,Bass EB , et al. The value of routine preoperative medical testing before cataract surgery. N Engl J Med 2000;342:168-175
Full Text | Web of Science | Medline2
Roizen MF . More preoperative assessment by physicians and less by laboratory tests. N Engl J Med 2000;342:204-205
Full Text | Web of Science | Medline
To the Editor:
Korn's recent discussion of scleroderma crisis omits mention of what was to my knowledge the first such case reversed by inhibition of angiotensin-converting enzyme.1 Management of that case, similar to the present one and also published in the Journal, 1 included measurement of plasma renin activity. The earlier report, of which I was an author, demonstrated the importance of increased activity of the renin–angiotensin system in the pathogenesis of scleroderma crisis. Since the initial episode 23 years ago,1 our patient has had two additional crises but is currently receiving angiotensin-converting–enzyme inhibitors, is normotensive (blood pressure, 120/80 mm Hg), has a serum creatinine concentration of 2.2 mg per deciliter,2 and works full time as a health care professional.
2 ReferencesJorge A. Lopez-Ovejero, M.D.
Cornell Medical School, New York, NY 100211
Lopez-Ovejero JA ,Saal SD ,D'Angelo WA ,Cheigh JS ,Stenzel KH ,Laragh JH . Reversal of vascular and renal crises of scleroderma by oral angiotensin-converting-enzyme blockade. N Engl J Med 1979;300:1417-1419
Full Text | Web of Science | Medline2
Lopez-Ovejero JA. A 20 year follow-up of the first renal and vascular crisis observed in scleroderma treated with an angiotensin converting enzyme inhibitor. In: Nicholls MG, Brunner HR, Ikram H, Sweet CS, Walker JF, eds. 100 years of the renin-angiotensin system. Whitehouse Station, N.J.: Merck, 1998:39-42.
Author/Editor Response
Dr. Korn replies:
To the Editor: The comments of O'Callaghan and colleagues highlight the potential utility of anti–PM-Sc1 antibodies in evaluation of patients with scleroderma and myositis. As noted, such antibodies may be found in patients with either scleroderma or the overlap syndrome of scleroderma and polymyositis. It is often a matter of preference whether one calls a patient with scleroderma who also has inflammatory myositis an “overlap” patient or a patient with scleroderma with myositis. I am not sure that the presence of the specific autoantibody necessarily aids in the designation.
Shapiro's comments on the use of alendronate in patients with scleroderma are cogent. Patients with scleroderma have delayed gastric emptying, impaired esophageal motility, and impaired function of the lower esophageal sphincter with severe reflux disease. The use of alendronate in such patients may increase the risk of esophageal ulceration. Parenteral bisphosphonates may be better tolerated.
Joseph H. Korn, M.D.
Boston University Medical Center, Boston, MA 02118-2526
jkorn@medicine.bu. edu
