Correspondence

Case 29-2002: Antiphospholipid-Antibody Syndrome

N Engl J Med 2003; 348:960-961March 6, 2003

Article

To the Editor:

We are told in Case 29-2002 (Sept. 19 issue)1 that the patient's urine contained 15 to 20 red cells per high-power field and 100 mg of protein per deciliter but that he did not have nephritis, yet a renal biopsy was not performed before the initiation of anticoagulant therapy. The diagnosis of systemic lupus erythematosus was considered but discarded because, among other factors, there was no nephritis.

The diagnosis of systemic lupus erythematosus was not made because only two or three criteria for the diagnosis were present,2 depending on whether or not the patient had arthritis, a line of reasoning that ignores the patient's obvious renal disease. In the absence of a renal biopsy, the pathogenesis of the renal disease is not known for sure, but given the presence of hypocomplementemia and antinuclear and anti-SM antibodies, an immune-complex–mediated nephritis may be strongly suspected. The American College of Rheumatology criteria for the diagnosis of systemic lupus erythematosus were established “for the purpose of identifying patients in clinical studies,” not for the purpose of making a clinical diagnosis, as in this case.

This boy was not treated for probable systemic lupus erythematosus and was certainly not treated as aggressively as would be appropriate if he had active lupus nephritis, as opposed to a pure coagulation nephropathy, for which warfarin therapy would be appropriate. A year later, while receiving warfarin, this 18-year-old still has “chronic microscopic hematuria and non–nephrotic-range proteinuria.” So far, his renal function is normal. A renal biopsy should be performed to help establish the most accurate diagnosis and thus the best course of treatment for his complicated illness.

Harvey E. Golden, M.D.
Rush Medical College, Chicago, IL 60612
hegolden@earthlink.net

2 References

1. 1

   Case Records of the Massachusetts General Hospital (Case 29-2002). N Engl J Med 2002;347:921-928
   Full Text | Web of Science | Medline

2. 2

   Tan EM, Cohen AS, Fries JF, et al. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 1982;25:1271-1277
   CrossRef | Web of Science | Medline

Author/Editor Response

The patient presented in cardiogenic shock and required emergency mitral-valve replacement. Renal biopsy was not indicated in that urgent clinical situation, on the basis of a single urine specimen containing protein and red cells.

Over the next 12 months, repeated urinalyses showed either no or very-low-level hematuria (1 or 2 red cells per high-power field). The urinary sediment contained no red cells or other casts. There was minimal proteinuria, with a ratio of spot urine protein to creatinine ranging from 0.15 to 0.16. The serum creatinine level was normal and stable at 0.8 mg per deciliter. The patient was not hypertensive. Under these circumstances, the likelihood of finding lupus nephritis was thought to be low. The risk associated with renal biopsy in the setting of an ongoing requirement for anticoagulation appeared to outweigh the potential benefit, in our opinion.

At the time of the last evaluation, the patient had arthritis, antiphospholipid antibodies, and antinuclear antibodies. He did not have hematologic abnormalities, neurologic disorders, or serositis. We did not have clinical evidence of immune-mediated disease that warranted aggressive treatment. We would use aggressive immunosuppressive therapy for class IV or V lupus nephritis,1 symptomatic neurologic disease, or vasculitis. The patient's arthritis had been managed with celecoxib and subsequently with prednisone, at a dose of 5 mg daily. We planned to monitor his renal status at intervals of three to six months. He has subsequently moved and has not returned for follow-up appointments, despite attempts to contact him.

David Steele, M.D.
John A. Mills, M.D.
Jonathan Kay, M.D.
Massachusetts General Hospital, Boston, MA 02114

1 References

1. 1

   Zimmerman R, Radhadkrishnan J, Valeri A, Appel G. Advances in the treatment of lupus nephritis. Annu Rev Med 2001;52:63-78
   CrossRef | Web of Science | Medline