Correspondence
Case 27-2003: A 36-Year-Old Man with Recurrent Epigastric Pain and Elevated Amylase Levels
N Engl J Med 2003; 349:2171-2172November 27, 2003
- Article
To the Editor:
In Case 27-2003 (Aug. 28 issue),1 a patient with two rare diseases — lymphoplasmacytic sclerosing pancreatitis and amyotrophic lateral sclerosis — is described. The pathological discussion concludes with the statement that these two diseases are unrelated, but as William Osler emphasized more than a century ago, the astute clinician should attempt to find a single explanation for all the symptoms. Could both illnesses have had a single underlying cause? Lymphoplasmacytic sclerosing pancreatitis has been described as autoimmune pancreatitis, and it has been suggested that autoimmunity is a cause of amyotrophic lateral sclerosis.2 Viruses can cause pancreatitis and are a possible cause of amyotrophic lateral sclerosis.3 It should be determined whether this unusual combination of illnesses has been diagnosed in other patients.
3 ReferencesAlbert B. Lowenfels, M.D.
New York Medical College, Valhalla, NY 10595
lowenfel@nymc.edu 1
Case Records of the Massachusetts General Hospital (Case 27-2003). N Engl J Med 2003;349:893-901
Full Text | Web of Science | Medline2
Appel SH ,Smith RG ,Alexianu MF ,Engelhardt JI ,Stefani E . Autoimmunity as an etiological factor in sporadic amyotrophic lateral sclerosis. Adv Neurol 1995;68:47-57
Web of Science | Medline3
Rowland LP ,Shneider NA . Amyotrophic lateral sclerosis. N Engl J Med 2001;344:1688-1700
Full Text | Web of Science | Medline
To the Editor:
I read with interest the recent case of a young man with probable autoimmune pancreatitis. I am an avid follower of the Case Records and am especially fond of the discussion building up to the diagnostic tests. I was surprised to read, however, that a Whipple procedure was considered diagnostic. If partial abdominal evisceration is thought to be diagnostic, then what procedure would be considered therapeutic?
Richard Makins, M.R.C.P.
Queen Mary's School of Medicine and Dentistry, London E1 2AD, United KingdomAuthor/Editor Response
Dr. Lowenfels is correct in pointing out the potential association between the two rare diseases affecting this patient. Current data indicate that lymphoplasmacytic sclerosing pancreatitis is an autoimmune disorder, and it has been linked to many other autoimmune diseases. Since the cause of amyotrophic lateral sclerosis is not known, and since autoimmunity may be an etiologic factor, one can certainly speculate that the two diseases are related. This association may be strengthened if similar cases are reported.
Dr. Makins remarks on the fact that the Whipple procedure was performed for diagnostic purposes. Perhaps it would have been more appropriate to label the operation a diagnostic–therapeutic procedure. Although lymphoplasmacytic sclerosing pancreatitis was suspected (and although the patient actually received treatment with corticosteroids for that reason), there was no definite diagnosis, and cancer remained a possibility. Furthermore, duodenopancreatectomy is often used in the treatment of patients with chronic pancreatitis and persistent pain, even when the treating physicians are completely certain of the diagnosis (which was not so in this case).
Carlos Fernández-del Castillo, M.D.
Massachusetts General Hospital, Boston, MA 02114
