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THE JOURNAL OF INFECTIOUS DISEASES VOL. 148, NO.2.

AUGUST 1983

Acquired Immune Deficiency Syndrome in the United States:


The First 1,000 Cases

Harold W. Jaffe, Dennis J. Bregman, and From the Acquired Immune Deficiency Syndrome Activity,
Richard M. Selik Center for Infectious Diseases, Centers for Disease Control,
Atlanta, Georgia

Between June 1981 and February 1983, the Centers for Disease Control (Atlanta) re-
ceived reports of 1,000 patients living in the United States who met a surveillance defi-
nition for the acquired immune deficiency syndrome (AIDS). Seventy-three percent of
these patients were diagnosed after January 1, 1982. The 1,000 patients included 284

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with Kaposi's sarcoma (KS), 497 with Pneumocystis carinii pneumonia (PCP), 83 with
KS and PCP, and 136 with opportunistic infections other than PCP. The overall mor-
tality has been 39.2%. Cases have been reported from 32 states and the District of
Columbia; New York, California, New Jersey, and Florida account for 82.7070 of the
reports. All but 61 of the patients could be classified into one or more of the following
groups: homosexual or bisexual men, intravenous drug abusers, Haitian natives, or pa-
tients with hemophilia. Epidemiologic trends in AIDS cases are consistent with the
gradual extension of an infectious agent into new populations.

In the spring of 1981, the Centers for Disease Cases and Methods
Control (CDC), Atlanta, received reports of the
unexpected occurrence of Pneumocystis carinii Case definition. For surveillance purposes,
pneumonia (PCP) and Kaposi's sarcoma (KS) AIDS is defined as the occurrence of biopsy-
among young homosexual men in California and proven KS and/or biopsy- or culture-proven infec-
New York City [1, 2]. These illnesses were asso- tion at least moderately predictive of cellular im-
ciated with an acquired cellular immunodeficiency mune deficiency (table 1). Patients who either had
of a type not previously described [3-5]. This im- received immunosuppressive therapy before the
mune disorder and the accompanying illnesses be- onset of illness or had preexisting illnesses asso-
came known as the acquired immune deficiency ciated with immunosuppression, such as congenit-
syndrome (AIDS). AIDS has been subsequently al immunodeficiency or lymphoreticular malig-
reported from other parts of the United States and nancy, are excluded. Also excluded are persons
among heterosexual men and women [6, 7]. In with KS who are over 60 years of age. Although
addition to PCP, patients with AIDS were found AIDS-like illness has been described in infants [8],
to be susceptible to a variety of other life-threaten- the present report is limited to patients who are at
ing opportunistic infections [5, 7]. The CDC be- least 10 years old. Immunologic testing is not re-
gan national surveillance for these diseases in June quired for inclusion of persons meeting the case
1981. By February 1983, 1,000 case reports had definition.
been received. This report summarizes the epi- Surveillance. AIDS surveillance has been both
demiologic features of the first 1,000 cases of retrospective and current. Active retrospective
AIDS reported from the United States. surveillance methods have been previously de-
scribed [7]. In brief, they included (1) review of
selected cancer tumor registries, (2) contact with
Received for publication May 3, 1983. selected physicians in 18 major metropolitan
We thank Paul Pinsky, Ann Rumph, and Jean Smith for as-
sistance with data analysis and David Auerbach, Mary Cham-
areas, and (3) review of requests received by the
berland, Selma Oritz, James Monroe, Pauline Thomas, and CDC's Parasitic Diseases Drug Service for penta-
the many other physicians and health department representa- midine isethionate. Current surveillance is pre-
tives who have assisted in the surveillance of acquired immune dominantly passive in nature, through receipt of
deficiency syndrome. reports from individual physicians and local or
Please address requests for reprints to Dr Harold W. Jaffe,
Acquired Immune Deficiency Syndrome Activity, Center for state health departments. These reports are sup-
Infectious Diseases, Centers for Disease Control, Atlanta, plemented by the active review of new requests for
Georgia 30333. pentamidine isethionate.

339
340 Jaffe, Bregman, and Selik

Table 1. Infections considered at least moderately CASES


70
predictive of underlying cellular immune deficiency.
60
A. Protozoal and helminthic infections 50
1. Cryptosporidiosis, intestinal, causing diarrhea for over 40
one month (on histology or stool microscopy) 30 Cases with Kaposi's sarcoma,
but not Pneumocvstis carinii pneumonia
2. Pneumocystis carinii pneumonia (on histology or on 20
microscopy of a "touch" preparation or bronchial 10
washings) o-!==F='F==t--.-~-+=~R=+==t=::I-W-+--+--I-+-l-l
3. Strongyloidosis, causing pneumonia, CNS infection, or 120
disseminated infection (on histology)
110
4. Toxoplasmosis, causing pneumonia or CNS infection (on 100
histology or microscopy of a "touch" preparation) 90

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B. Fungal infections 80
1. Aspergillosis, causing CNS or disseminated infection (on
70
culture or histology) 60
2. Candidiasis, causing esophagitis (on histology, 50
microscopy of a "wet" preparation from the esophagus, 40
or endoscopic findings of white plaques on an 30 Cases with Pneumocystis carinii pneumonia,
erythematous mucosal base) 20
but not Kaposi's sarcoma

3. Cryptococcosis, causing pulmonary, CNS, or 10


disseminated infection (on culture, antigen detection, O-+-"""T"""""T""................-f"'=t-.......,....-+-+-+-+-+-+-+-+---j.---j.~~~
histology, or India ink preparation of CSF)
C. Bacterial infection 30
20 Cases with both Kaposi's sarcoma
1. "Atypical" mycobacteriosis (species other than tuber-
and Pneumocystis carinii pneumonia
culosis or lepra), causing disseminated infection (on 10
culture) O-+-"""T""-.--.--r........................---r--F=t--+-+-+--+--+---4---4~~--l
D. Viral infection
40
1. Cytomegalovirus, causing pulmonary, gastrointestinal
30 Cases with neither Kaposis sarcoma
tract, or CNS infection (on histology) nor Pneumocystis carinii pneumonia,
20
2. Herpes simplex virus, causing chronic mucocutaneous in- but some other opportunistic
10 infection
fection with ulcers persisting more than one month or
O-+--r=-f":=t-7'T-r:::-f"""'I--:--F=F===l---F=F=J.--+-+~---4---4---4--l
pulmonary, gastrointestinal tract, or disseminated infec- 2 3 4
tion (on culture, histology, or cytology) 1982
3. Progressive multifocal leukoencephalopathy (presumed Quarter of Diagnosis

to be caused by papovavirus) (on histology)


Figure 1. The first 1,000 reported cases of AIDS in the
NOTE. Within each category, the diseases are listed in United States, by quarter of diagnosis, for each of four
alphabetical order. "Disseminated infection" refers to involve- mutually exclusive disease groups.
ment of lungs and multiple lymph nodes. The required diagnostic
methods with positive results are shown in parentheses. insignificant. Confidence intervals following a
statistic represent a two-tailed interval with 2.5070
Statistical techniques. The incidence of AIDS in each tail.
was examined using a least-squares linear regres-
sion [9] of the natural log of the exponential func-
Results
tion Y = aebx (a > 0), where Y = number of
cases and x = calendar quarter since the first AIDS appears to be a new illness of rapidly in-
quarter of 1978. The estimated value of the slope creasing incidence (figure 1). Retrospective sur-
(b) was taken to represent the case reporting rate. veillance established that 77 patients meeting our
The ratio of the case reporting rate for PCP to the case definition had been diagnosed before the first
rate for KS cases was interpreted as the relative cases were reported in the spring of 1981. The
reporting rate. earliest KS cases were diagnosed in the first
Other statistical methods included the Z test for quarter of 1978, while the first PCP case was diag-
differences between proportions [9], the odds nosed in the second quarter of 1979. Seventy-three
ratio for a single 2 X 2 table with a Taylor series percent of the first 1,000 cases have been diag-
confidence limit [10], and the X2 test of indepen- nosed since January 1, 1982. The rates of increase
dence for 2 x 2 tables [9]. The acceptable type I for patients reported to have KS only and PCP
error rate was taken to be 0.05. Results whose test only are approximated by exponential curves with
statistic revealed a higher error rate were treated as PCP cases reported at a 20070 faster rate than KS
First 1,000 Cases of AIDS 341

Table 2. Distribution and mortality of AIDS by disease San Francisco, Los Angeles, Newark, and Miami.
group. Comparing the geographic distribution of the first
No. of No. of 250 reported cases to the last 250 cases (table 3),
Disease group cases deaths Mortality (Olo)* one finds that the proportion of cases reported
KS but not PCP 284 61 21.5 from New York State has significantly decreased
PCP but not KS 497 230 46.3 (P < 0.05), while there has been a corresponding
KS and PCP 83 40 48.2 increase in reported cases from New Jersey and
Othert 136 61 44.8 other states.
Total 1,000 392 39.2 All but 61 of the 1,000 cases could be classified
* Percentage of patients reported to have died, as of January into one or more of the following risk groups:
31, 1983, regardless of when AIDS was diagnosed. homosexual or bisexual men, iv drug abusers,

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t Neither KS nor PCP, but some other opportunistic infection. Haitians living in the United States, or patients
with hemophilia. As shown in a schematic Venn
cases. The apparent small decrease in patients with diagram (figure 3), these risk groups were not
both KS and PCP and with opportunistic infec- mutually exclusive. The largest overlap between
tions other than PCP during the last quarter of risk groups was for homosexual or bisexual men
1982 is probably a result of the lag time between and iv drug abusers. Of the 882 patients who be-
diagnosis and receipt of a case report. longed to one of these groups, 81 (9.2%) belonged
Mortality from AIDS has been high (table 2). to both. Only rarely did Haitians or hemophiliacs
The overall crude mortality of 39.2010 is an under- intersect with other risk groups.
estimate of the true mortality because it does not To simplify data analysis, the 1,000 cases were
consider that most cases have been diagnosed re- reclassified into a hierarchy of mutually exclusive
cently and have not been followed long enough to risk groups. Arbitrarily, homosexual or bisexual
reasonably assess outcome. Of the 269 AIDS pa- men were placed first in the hierarchy whether or
tients diagnosed before January 1, 1982, 196 not they had other risk factors. The second group
(73%) are reported to have died. consisted of iv drug abusers without a history of
Cases have been reported from 32 states and the male homosexuality (either heterosexual or of un-
District of Columbia (figure 2). The states of New known sexual orientation). The third group con-
York, California, New Jersey, and Florida ac- sisted of persons of Haitian origin without a his-
counted for 827 of the 1,000 cases. Within these tory of male homosexuality or iv drug abuse. The
states, the great majority of cases were reported fourth group included persons with hemophilia in
from major metropolitan areas: New York City, none of the previously mentioned groups, and the

~lt~\t~NumberOf
rn
Cases .200-500
60-70
~ 10-25
1-9
D None

Figure 2. Distribution of AIDS by state of residence at onset of illness (asterisks refer to standard metropolitan
statistical areas).
342 Jaffe, Bregman, and Selik

Table 3. Distribution of AIDS by state of residence for each consecutively reported set of 250 cases.
Total
State of First 250 Second 250 Third 250 Fourth 250 first 1,000
residence reported cases (070) reported cases (070) reported cases (070) reported cases (070) reported cases (070)
California 24.0 18.0 19.6 25.2 21.7
Florida 4.4 10.0 5.6 4.0 6.0
New Jersey 4.4 7.6 7.2 7.6 6.7
New York 53.2 48.0 48.8 43.2 48.3
Other 14.0 16.4 18.8 20.0 17.3

fifth group consisted of persons in none of the to be slightly younger than the homosexual or bi-

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other four groups. sexual men. Of the 59 female patients, 49.2% were
Within this hierarchial classification, homo- iv drug abusers. Compared with the homosexual
sexual or bisexual men accounted for 727 of the or bisexual male patients, the iv-drug-abusing pa-
1,000 cases. Compared with the iv drug abusers, tients were 6.9 (4.7 to 10.3) times more likely to be
Haitians, and persons with hemophilia, homo- either black or Hispanic.
sexual or bisexual patients were 22.9 (11.1 to 47.2) The distribution of cases by mutually exclusive
times more likely to have KS (table 4). Only 3.8010 risk groups has changed over time (table 7). Be-
of the patients belonging to these three hetero- tween the first and second sets of 250 reported
sexual risk groups had KS, with or without PCP. cases, the proportion of homosexual or bisexual
The distribution of cases by state of residence men decreased significantly (P < 0.001), while
varied among the risk groups (table 5). Almost there was a corresponding increase in the propor-
three-quarters of the homosexual or bisexual male tion of iv drug abusers, Haitians, and hemophiliac
patients were from either New York or California, patients. From the first to the last set of 250 cases,
while about 90010 of the cases among iv drug the proportion belonging to no identified risk
abusers were from either New York or New group has doubled.
Jersey. The Haitian patients tended to live in The 61 patients who seem not to belong to any
either Florida or New York; the patients with of the recognized risk groups (tables 4-6) are
hemophilia did not live in the states which ac- under investigation to determine whether new
counted for most of the cases from other risk population groups are at risk for AIDS. Unfor-
groups. tunately, information regarding risk factors is in-
Demographic characteristics of cases by mu- adequate for some of these patients because they
tually exclusive risk groups are shown in table 6. have died or cannot be interviewed at the time they
Although patients ranged in age from 10 to 73 are reported. Another portion of the 61 patients
years, 47.9% were 30-39 years old. The Haitian probably represents the expected "background"
patients and patients who abused iv drugs tended occurrence of KS- that is, disease not associated

HEMOPHILIACS: 8
~ INTRAVENOUS DRUG
ABUSERS: 236
/

(0
NONE OF
61 -THE OTHER Figure 3. Overlap of groups at in-
GROUPS: 61 creased risk for AIDS.

-HAITIANS: 54

HOMOSEXUAL OR
BISEXUAL MEN: 727
First 1,000 Cases of AIDS 343

Table 4. Distribution of AIDS by disease group for each risk group.


Distribution by disease group (070)
Risk group* No. of cases KS but not PCP PCP but not KS KS and PCP Othert
Homosexual or bisexual men 727 36.0 43.7 11.1 9.1
Intravenous drug abusers 155 2.6 78.1 1.3 18.1
Haitians 50 4.0 38.0 0 58.0
Hemophiliacs 7 0 85.7 0 14.3
Others 61 26.2 54.1 0 19.7
Total 1,000 28.4 49.7 8.3 13.6
* Referring to a hierarchy of mutually exclusive risk groups, as described in the text.

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t Neither KS nor PCP, but some other opportunistic infection.

with AIDS. Several new risk groups may, how- tions, it has established that AIDS is a rapidly
ever, be emerging from the study of these 61 pa- emerging, highly serious health problem in the
tients. For example, five of the 61 patients are United States. Mortality has been very high.
women with PCP who deny iv drug abuse them- Earlier diagnosis and treatment of KS and the op-
selves but who have been steady sexual partners of portunistic infections may improve survival. How-
male iv drug abusers. One of these men had AIDS ever, spontaneous return of normal immune func-
himself. Five other patients with no apparent risk tion has not been reported in AIDS patients who
factors were reported to have received a trans- met the CDC case definition. Until a therapy to
fusion of blood components within three years 'of reverse the immune dysfunction of AIDS becomes
the onset of their illness. The donors of the blood available, mortality is likely to remain high.
components received by these patients are now The occurrence of AIDS among the diverse
under investigation. population groups described in this report sug-
gests an infectious etiology. Reports of AIDS
among the sexual partners of homosexual and
Discussion
heterosexual AIDS patients are consistent with
Because of the predominantly passive nature of transmission of a putative "AIDS agent" by sexual
the CDC's present AIDS surveillance systems, the or other intimate person-to-person contact [11,
results presented in this report must be interpreted 12]. Transmission of such an agent through blood
cautiously. Some AIDS cases may not be diag- or blood products is consistent with the occur-
nosed, and others may be diagnosed but not re- rence of AIDS in iv drug abusers, patients with
ported. These biases may vary over time and from hemophilia, and transfusion recipients.
place to place. Active AIDS surveillance in The overlap between the homosexual or bi-
selected New York City hospitals and among pa- sexual male risk group and the iv drug abuser
tients with hemophilia is underway; programs are group might explain spread of an "AIDS agent"
being implemented by the CDC in collaboration from one group to the other. Geographically, this
with the New York City Health Department and overlap occurs particularly in New York City and
the National Hemophilia Foundation. northern New Jersey.
Although the present surveillance has limita- Relatively little overlap between the Haitian

Table 5. Distribution of AIDS by state of residence for each risk group.


Distribution by state of residence (OJo)

Risk group* No. of cases California Florida New Jersey New York Others

Homosexual or bisexual men 727 27.9 4.1 4.1 45.8 18.0


Intravenous drug abusers 155 3.9 1.3 19.4 70.3 5.1
Haitians 50 2.0 48.0 4.0 34.0 12.0
Hemophiliacs 7 0 0 0 0 100
Others 61 11.5 6.6 8.2 39.3 34.4
Total 1,000 21.7 6.0 6.7 48.3 17.3

* Referring to a hierarchy of mutually exclusive risk groups, as described in the text.


344 Jaffe, Bregman, and Selik

Table 6. Demographic characteristics of AIDS by risk group.


Distribution Distribution by race/ethnicity (0/0)
by sex (%)
No. of Median age in White, Black,
Risk group* cases years (range) Male Female non-Hispanic non-Hispanic Hispanic Other Unknown
Homosexual or
bisexual men 727 35 (19-73) 100 0 71.9 16.9 10.4 0.1 0.6
Intravenous
drug abusers 155 33 (20-53) 81.3 18.7 23.9 47.7 27.7 0.6 0.6
Haitians 50 30 (19-54) 90.0 10.0 2.0 98.0 0 0 0
Hemophiliacs 7 49 (10-59) 100 0 100 0 0 0 0
Others 61 33 (15-64) 59.0 41.0 34.4 41.0 18.0 4.9 1.6

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Total 1,000 34 (10-73) 94.1 5.9 58.8 27.1 12.9 0.5 0.6
* Referring to a hierarchy of mutually exclusive risk groups, as described in the text.

group and other risk groups has been docu- other population groups and in other parts of the
mented. Illnesses indicative of AIDS have oc- United States. Although these passive surveillance
curred in Haitians living in Haiti, and some data must be interpreted cautiously, the trends are
Haitians diagnosed in the United States became ill consistent with the gradual extension of an infec-
before leaving Haiti [13] (AIDS Activity, CDC, tious agent into new populations.
unpublished observations). Whether other Haitians Although we believe that the diseases included
living in the United States acquired their disease in the CDC surveillance definition are at least
here or in Haiti is unknown. moderately predictive of acquired cellular immune
Although a single-infectious-agent hypothesis deficiency, the spectrum of AIDS may not be
would seem to explain much of the AIDS epi- limited to KS and opportunistic infections. Other
demic, certain epidemiologic observations remain unusual malignancies, including both a lymphoma
puzzling. For example, what explains the excess of classified as either Burkitt's lymphoma or diffuse
KS cases among homosexual or bisexual men com- undifferentiated non-Hodgkin's lymphoma [14,
pared with other AIDS risk groups? Perhaps the 15] and lymphoma limited to the brain [16], have
route of transmission (sexual vs nonsexual) of an been reported among young homosexual men,
"AIDS agent" into a susceptible host plays a role with or without previously described manifesta-
in determining clinical outcome. Or perhaps a tions of AIDS. Autoimmune thrombocytopenia
cofactor present in the homosexual male popula- associated with impaired cellular immunity has
tion, such as repeated exposure to cytomegalo- been described among both homosexual men and
virus or use of inhalant sexual stimulants, predis- patients with hemophilia [17, 18]. Physicians from
poses homosexual AIDS patients to develop KS. several major metropolitan areas in the United
The epidemiologic trends presented here suggest States have reported the occurrence of chronic,
that AIDS, a disease first recognized among generalized, unexplained lymphadenopathy in
homosexual men in California and New York homosexual men [19]. Many of these patients have
City, is now becoming an increasing problem in laboratory evidence of cellular immunodeficiency,

Table 7. Distribution of AIDS by mutually exclusive risk groups for each consecutively reported set of 250 cases.
Total first
First 250 Second 250 Third 250 Fourth 250 1,000 reported
Risk group* reported cases (%) reported cases (%) reported cases (%) reported cases (%) cases (%)
Homosexual and
bisexual men 83.6 68.0 71.2 68.0 72.7
Intravenous
drug abusers 9.6 18.4 14.8 19.2 15.5
Haitians 2.4 9.6 5.2 2.8 5.0
Hemophiliacs 0 0.8 0.8 1.2 0.7
Others 4.4 3.2 8.0 8.8 6.1
* Referring to a hierarchy of mutually exclusive risk groups, as described in the text.
First 1,000 Cases of AIDS 345

and a few have developed life-threatening mani- and Pneumocystis carinii pneumonia among homosex-
festations of AIDS [20] (AIDS Activity, unpub- ual male residents of Los Angeles and Orange Counties,
California. MMWR 1982;31:305-7
lished observations). Finally, abnormalities of
12. Centers for Disease Control. Immunodeficiency among
cell-mediated immunity have been noted in both female sexual partners of males with acquired immune
asymptomatic homosexual men and patients with deficiency syndrome (AIDS)-New York. MMWR
hemophilia [21-25]. The meaning of this "asymp- 1983;31:697-8
tomatic immunosuppression" is not clear. It may 13. Pitchenik AE, Fischl MA, Dickinson GM, Becker OM,
Fournier AM, O'Connell MT, Colton RM, Spira TJ.
represent a mild manifestation in the AIDS spec-
Opportunistic infections and Kaposi's sarcoma among
trum or may be an unrelated phenomenon. Haitians: evidence of a new acquired immunodeficiency
Continuing surveillance will be needed to de- state. Ann Intern Med 1983;98:277-84
termine whether AIDS will continue to increase in 14. Doll DC, List AF. Burkitt's lymphoma in a homosexual

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