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LETTERS TO THE EDITOR 473

Wedgewood that not only did we apply the

ac-tuarial analysis to our own experience in iupus

erythematosus but piior to that we also used it

to study the outcome of chronic ulcerative coli-tis in children.’

G. B. STIciuER, M.D.

E. C. BURKE, M.D.

REFERENCES

1. Meislin, A. G., and Rothfield, N. : Systemic

lupus erythematosus in childhood : Analysis of

42 cases, with comparative data on 200 adult

cases followed concurrently. PEDIATRICS, 42: 37, 1968.

2. Hagge, W. W., Burke, E. C., and Stickler,

C. B.: Treatment of systemic lupus

erythe-matosus complicated by nephritis in children.

PEDIATRICS, 40:822, 1967.

3. Good, R. A., Venters, H., Page, A. R., and

Good, T. A. : Diffuse connective tissue

dis-eases in childhood with a special comment on

connective tissue diseases in patients with

agammaglobulinemia. J. Lancet, 81:192,

1961.

4. Michener, \V. M., Gage, R. P., Saner, W. G.,

and Stickler, G. B. : The prognosis of chronic

ulcerative colitis in children. New Eng. J.

Med., 265:1075, 1961.

EDIToR’s NOTE : Doctors Meislin and

Roth-field comment as follows:

So long as the diagnosis of SLE is based on

an overall view of the entire clinical picture

with assistance from various laboratory tests

such as LE cells, antinuclear antibodies, and

skin and renal biopsies, there will be problems

in arrivmg at a definitive diagnosis and the

in-evitable semantic arguments” will continue to

arise. We welcome Drs. Stickler and Burke’s

letter, which provides the opportunity to fur-tlier clarify our concept of SLE.

Criteria for diagnosis of SLE were set forth

in our second paragraph of the section entitled

Discussion,1 in as precise a manner as we feel

tile disease permits at the present state of

knowledge. We further stated, “all patients

fulfilled tile criteria set forth by Dubois.”2

In-deed, our criteria were more restrictive than

Dubois’ since we specifically included multiple

system involvement as a prerequisite for

diag-nosis and inclusion in our series. This we feel

automatically excluded cases of RA with

posi-tive LE cell test and discoid LE with positive LE cell test (though three cases of discoid lupus which evolved into SLE were included in our series).

Our concept of “rheumatoid arthritis with a

positive LE cell test” is not firm, and we

ap-proach tile entire subject with profound

humil-ity. Judging from the frequently reported

de-layed appearance of multi-system involvements

and the frequently delayed emergence of SLE,

it would not surprise us if this entity were to

become another of the many faces of SLE.

Nonetheless, we agree with Dubois and with

the correspondents that it is important to keep

this group separate and excluded from SLE, as

we have done. Perhaps renal biopsies would be

helpful in resolving the nature of this problem

as suggested by the work of Poilak, et al. and

Dubois alld n’5

We deliberately avoided the term,

drug-in-duced lupus, employing drug-associated or

drug-related disease instead, since we are not

certain of the etiologic relationship, something

we speculated upon in our article. Four cases

of possible drug-related disease were included

in the series (three treated with INH and one

On anti-convulsants

)

. Three of these four

expe-rienced progressive active disease, two with

fatal outcomes, following discontinuation of

drug therapy as stated in our discussion. This

represents a much graver prognosis than

cx-pected from “drug induced lupus,”0 indeed it

is a poorer prognosis than we observed in the

non-drug related disease.

Seven of 39 patients had BUN elevation by

the time of diagnosis and renal disease was

considered present in 22 of 42 patients at

diag-nosis based upon urinalyses. The overall

mci-dence of renal disease was 29 of 41 cases, or

71% of cases followed. This incidence is lower

than that reported in most pediatric series

(Table I) . Variation exists and the published

series are small, but it is remarkable that four

of the six series indicate renal involvement

within relatively narrow limits of 71 to 82% of

cases. Noteworthy are the different bases for

defining renal disease-urinalysis, Addis count,

renal function tests, and renal biopsies-which appear to be relevant to the incidence of renal disease reported.

The incidence of renal involvement in repre-sentative adult series of SLE varies from 46.1% to 65t.8 Our own experience in a previous study

showed renal disease in 29 of 52 lupus patients

(56%) ages 8 to 80, all present at the first

ob-servation,9 and in our current studyl 51 of

200 adults had lupus nephritis. Though age

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.lge (yr)

5-13

5-15 3-15 6-19 2-16

<15

4-18

<14 <15 1

25

‘27

TABLE 1

INcIl)EN(E OF RF:N%I. INVOLvEMENT IN (‘iiiimioon SLE

‘Vu,,,ber ,

Renal c 11(0(11

of . . t 010 iiu’nl.v

- Dtsea.c Dixea.se

Patzen1.

11 5 451 Lower incidence than heart or lung illvolvelnent.

15 10 67% Throughout course.

41 ‘29 7l/ ‘2’2/4 (5’2%) at onset; based on clinical findings.

‘21 15 71% 56/9() in total series, ages 6-70.

30 3 77% Renal involvenient questionable in four cases

with no significant proteinuria.

.5’2 Inclusive of the 35 cases repoited in 1963,6 vlich iflclude(l seven cases of “drug-induced liipus.”

18 Characteristic cases of SLE with uiiiforni clinical iIfl(l lal) pi(ture.

37 33 89’

11 ii looc/2) l4nse(l on 1)iol)sies and electron Illicroscopic

studies; three of 1 1 ilad nornial uriiies

474 LETTERS TO THE EDITOR

indicated 110 correlation between age and

in-cidence of renal disease.bo

The correspondents’ criteria for diagnosis”

are identical with our own, except that lOfYt

of their patients had positive LE preps and

ESR elevation while our figures were 90% and

80%, respectively, by the time of diagnosis. Of

the four patients in our series with negative LE

preps at diagnosis, three subsequently

devel-oped positive LE preps, and one had skin,

sub-cutaneous nodule, and renal biopsies

compat-ible with SLE, strongly positive ANA, and died

within 6 months of diagnosis of renal failure

and pneumonia. All had serious multisystem

disease and indicate to US tile propriety of

mak-illg the diagnosis of SLE in the absence of a

Positive LE prep, if other overwhelming

cvi-dence of the disease is present. Newer

tech-niques, such as antinuclear antibody

determina-tiOIls, may enhance the reliability of diagnosis,

but complete accuracy awaits the isolation of

an etiological agent.

We are therefore compelled to dispute the

statement that we are presenting “data on a

group of diseases considered to be SLE.” What

we may very well be doing is broadening the

clinical spectrum of juvenile SLE as seen by

Drs. Stickler and Burke. With the description of the LE cell phenomenon at the Mayo Clinic

in 1948,12 the concept of SLE was enlarged

from a rare, fulminating disorder of

pleo-morphic nature. Our study simply supports the

case against the more restricted concept of

childhood SLE as a generally rapidly fatal

dis-ease, almost invariably associated with severe

renal disease.

Our statement that proteinuria and hematu-na were present in “most patients’ applied to

a discussion of patients with renal disease and

not to the entire series. We apologize for the

ambiguity.

All attempt should be made to explain tile

differences observed in childhood SLE in New

York and Minnesota. We cannot exclude the

possibility of geographical differences, and the

entire ecology of SLE bears illvestigation. For example, the incidence of SLE in Rochester,

Minnesota, has been reported to be greater

tilall ill New York City or Sweden;1’ and its

in-cidence in England is lower than ill the United

States.” Also, racial differences may exist.”

Almost half of our series were non-Caucasian. A point must also be made for geographical

and institutiollal i)ias. A large, rural, referral

center will attract more seriously ill patients

with poorer prognosis, whereas the urban mcd-ical center such as our OW with its

surround-ing dense population viIl draw a broader

spec-trum of freshly diagnosed disease-from

be-nign to rapidly fatal-though criteria for

diag-nosis remam the same.

Therapy was described in detail with

spe-cific dosage schedules under the heading

enti-tied “Treatment.” Empirical therapy was that dosage sufficient to control disease activity. All

patients were treated with prednisone, most

with antimalarials as well; therefore, we were

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LETTERS TO THE EDITOR 475

the disease. At present, our clinical impression is that long-term chioroquine treatment is

help-ful in controlling disease activity. Since no

con-trolled studies were done, we cannot offer

ob-jective evidence for this impression. Analysis of

chloroquine effect is currently under study in

patients whose drug was discontinued due to

ocular toxicity.”

Actuarial treatmeilt of the data and

compu-tatioll of life tables and survival rates are not

lldW to the medical literature” nor to SLE.””

However, we feel that construction of survival

models based on a numerically small

experi-ence renders the data more intelligible than

re-porting raw survival probai)ihties based on

similar numbers. The models also permit more

meaningful graphic comparison between series

of patients when survival data is treated in the

same way.

AARON G. MEISLIN, M.D.

NAOMI ROTHFIELD, M.D.

REFERENCES

1. Meislill, A. G., and Rothfield, N. : Systemic

lupus erythematosus in childhood. Analysis

of 42 cases, Witil comparative data on 200

adult cases followed concurrently.

PEDIAT-RICS, 42:37, 1968.

2. Dubois, E. L. : Lupus Erythematosus. New

York: McGraw-hill Inc., 1966.

3. Kievitz, J. H., Gosiings, J., Shuit, H., and

Hijmans, W. : Rheumatoid arthritis and the

positive L.E. cell phenomenon. Ann. Rileum. Dis., 15:211, 1956.

4. Pollak, V. E., Pirani, C. L., Steck, I. E., and

Kark, R. M. : The kidney in rheumatoid ar-thritis: Studies by renal biopsy. Arthritis

Rheum., 5:1, 1962.

5. Berman, L. B., and Dubois, E. L. : Renal

biopsy in rheumatoid arthritis with L. E.

cells. (Ahst.) Arthritis Rheum., 5:283, 1962.

6. Jacobs, J.: Systemic lupus erythematosus in

childhood. PEDIATRICS, 32:257, 1963.

7. Dubois, E. L., and Tuffanelli, D. : Clinical

manifestations of systemic Itipus

erythemato-5115. J.A.M.A., 190:104, 1964.

8. lIarvey, A. I., Shulman, L. E., Tumult, A.,

Conley, C. L., and Schoenricll, E. H. :

Svs-temic lupus erytllenlatOsus : Review of the

literature and clinical analysis of 138 cases.

Medicine, 33:291, 1954.

9. Rothfleld, N., McCluskey, H. T., and Baldwin,

I). S. : Renal disease in systemic lupus

er\-thematosus. New Eng. J. Med., 269:537,

196.3.

10. Urowitz, M. B., Stevens, M. B., and Shulnlan,

L. E. : The influence of age of the clinical Pattern of systemic lupus erythematosus.

(Abst.) Arthritis Rheum., 10:319, 1967.

11. Hagge, \V. W., Burke, E. C., and Stickler,

G. B.: Treatment of systemic lupus

erythe-matosus complicated by nephritis in

chil-dren. PEDIATRICS, 40:822, 1967.

12. Hargraves, M., Richmond, H., and Morton, R.:

Presentation of two bone marrow elements:

The “tart” cell and L. E. Cell. Proc. Staff

Meet. Mayo Clin., 23:25, 1948.

13. Nobrega, F. T., Ferguson, R. H., Kurland, L. T.,

and Hargraves, M. : Population studies of

the rheumatic diseases. In Bennett, P. II.,

and Wood, P. H. N., ed. : Exerpta Medica

Foundation, 1968.

14. Bywaters, E. G. L., and Scott, J. T. : Progress

in clinical rheumatology. in Dixon, A. S. J.,

ed. : Systemic Disease of Connective Tissues.

Boston: Little, Brown and Company, p. 130, 1964.

15. Siegel, M., and Lee, S. L. : The epidemology

of systemic lupus erythematosus: Results of

a population study in New York City.

Pro-ceedings of the Third International

Sympo-sium on Population Studies of the

Rheu-inatic Diesases, New York, June 1966.

16. Rothfield, N. : Unpublished data.

17. Berkson, J., and Gage, R. P.: Calculation of

survival rates for cancer. Proc. Staff Meet. Mayo Clin., 25:270, 1950.

18. Merrell, M., and Shulman, L. E. :

Determina-tion of prognosis in chronic disease illu5-trated by SLE. J. Chronic Dis., 1 : 12, 1955. 19. Kellem, R. E., and Haserick, J. R.: SLE-A

statistical evaluation of mortality’ based on a

consecutive series of 299 patients. Arch. In-tern. Med., 113:92, 1964.

20. Zetterstroni, R., and Bergiund, C. : Systemic lupus erythematosus in childhood: a clinical

study. Acta Paediat. ( Stockholm), 45:189,

1956.

21. Grihetz, D., and Henley, W.: Systemic lupus

erythematosus in childhood. J. Mount Sinai

Hosp. N.Y., 26:289, 1959.

22. Soffer, L. J., Southren, A. L., Weiner, H. E.,

and Wolf, R. L. : Renal manifestations of

systemic lupus erytheniatosus : a clinical and

pathologic study of 90 cases. Ann. Intern.

Med., 54:215, 1961.

23. Hanson, V., and Kornreich, H. : Systemic

rheu-nlatic disorders in childhood. Bull. Rheum.

Dis., 17:435, 1967.

24. Jacobs, J. C. : Lupus erythematosus with

ne-phritis. PEDIATRICS, 42:369, 1968.

25. Peterson, R. D. A., Vernier, R. L., and Good,

R. A. : Lupus erythematosus. Pediat. Clin. N. Amer., 10:941, 196,3.

26. Cook, C. D., Wedgwood, R. J. P., Craig, J. M.,

Ilartmann, J. R., and Janeway, C. A.:

Svs-temic inpus erytllenlatosus. Description of

37 cases in children and a discussion of

endocrine tilerapy in 32 of the cases.

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1969;43;473

Pediatrics

Aaron G. Meislin and Naomi Rothfield

Letter to the Editor

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1969;43;473

Pediatrics

Aaron G. Meislin and Naomi Rothfield

Letter to the Editor

http://pediatrics.aappublications.org/content/43/3/473

the World Wide Web at:

The online version of this article, along with updated information and services, is located on

American Academy of Pediatrics. All rights reserved. Print ISSN: 1073-0397.

References

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