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

NOTE. Within each category, the diseases are listed in
alphabetical order. "Disseminated infection" refers to involve-
ment of lungs and multiple lymph nodes. The required diagnostic
methods with positive results are shown in parentheses.
Statistical techniques. The incidence of AIDS
was examined using a least-squares linear regres-
sion [9] of the natural log of the exponential func-
tion Y = aebx (a > 0), where Y = number of
cases and x = calendar quarter since the first
quarter of 1978. The estimated value of the slope
(b) was taken to represent the case reporting rate.
The ratio of the case reporting rate for PCP to the
rate for KS cases was interpreted as the relative
reporting rate.
Other statistical methods included the Z test for
differences between proportions [9], the odds
ratio for a single 2 X 2 table with a Taylor series
confidence limit [10], and the X2 test of indepen-
dence for 2 x 2 tables [9]. The acceptable type I
error rate was taken to be 0.05. Results whose test
statistic revealed a higher error rate were treated as
Figure 1. The first 1,000 reported cases of AIDS in the
United States, by quarter of diagnosis, for each of four
mutually exclusive disease groups.
insignificant. Confidence intervals following a
statistic represent a two-tailed interval with 2.5070
in each tail.
Results
AIDS appears to be a new illness of rapidly in-
creasing incidence (figure 1). Retrospective sur-
veillance established that 77 patients meeting our
case definition had been diagnosed before the first
cases were reported in the spring of 1981. The
earliest KS cases were diagnosed in the first
quarter of 1978, while the first PCP case was diag-
nosed in the second quarter of 1979. Seventy-three
percent of the first 1,000 cases have been diag-
nosed since January 1, 1982. The rates of increase
for patients reported to have KS only and PCP
only are approximated by exponential curves with
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.
cases. The apparent small decrease in patients with
both KS and PCP and with opportunistic infec-
tions other than PCP during the last quarter of
1982 is probably a result of the lag time between
diagnosis and receipt of a case report.
Mortality from AIDS has been high (table 2).
The overall crude mortality of 39.2010 is an under-
estimate of the true mortality because it does not
consider that most cases have been diagnosed re-
cently and have not been followed long enough to
reasonably assess outcome. Of the 269 AIDS pa-
tients diagnosed before January 1, 1982, 196
(73%) are reported to have died.
Cases have been reported from 32 states and the
District of Columbia (figure 2). The states of New
York, California, New Jersey, and Florida ac-
counted for 827 of the 1,000 cases. Within these
states, the great majority of cases were reported
from major metropolitan areas: New York City,
Haitians living in the United States, or patients
with hemophilia. As shown in a schematic Venn
diagram (figure 3), these risk groups were not
mutually exclusive. The largest overlap between
risk groups was for homosexual or bisexual men
and iv drug abusers. Of the 882 patients who be-
longed to one of these groups, 81 (9.2%) belonged
to both. Only rarely did Haitians or hemophiliacs
intersect with other risk groups.
To simplify data analysis, the 1,000 cases were
reclassified into a hierarchy of mutually exclusive
risk groups. Arbitrarily, homosexual or bisexual
men were placed first in the hierarchy whether or
not they had other risk factors. The second group
consisted of iv drug abusers without a history of
male homosexuality (either heterosexual or of un-
known sexual orientation). The third group con-
sisted of persons of Haitian origin without a his-
tory of male homosexuality or iv drug abuse. The
fourth group included persons with hemophilia in
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.
Within this hierarchial classification, homo-
sexual or bisexual men accounted for 727 of the
1,000 cases. Compared with the iv drug abusers,
Haitians, and persons with hemophilia, homo-
sexual or bisexual patients were 22.9 (11.1 to 47.2)
times more likely to have KS (table 4). Only 3.8010
of the patients belonging to these three hetero-
sexual risk groups had KS, with or without PCP.
The distribution of cases by state of residence
varied among the risk groups (table 5). Almost
three-quarters of the homosexual or bisexual male
patients were from either New York or California,
while about 90010 of the cases among iv drug
abusers were from either New York or New
Jersey. The Haitian patients tended to live in
either Florida or New York; the patients with
hemophilia did not live in the states which ac-
counted for most of the cases from other risk
groups.
Demographic characteristics of cases by mu-
tually exclusive risk groups are shown in table 6.
Although patients ranged in age from 10 to 73
years, 47.9% were 30-39 years old. The Haitian
patients and patients who abused iv drugs tended
sexual men. Of the 59 female patients, 49.2% were
iv drug abusers. Compared with the homosexual
or bisexual male patients, the iv-drug-abusing pa-
tients were 6.9 (4.7 to 10.3) times more likely to be
either black or Hispanic.
The distribution of cases by mutually exclusive
risk groups has changed over time (table 7). Be-
tween the first and second sets of 250 reported
cases, the proportion of homosexual or bisexual
men decreased significantly (P < 0.001), while
there was a corresponding increase in the propor-
tion of iv drug abusers, Haitians, and hemophiliac
patients. From the first to the last set of 250 cases,
the proportion belonging to no identified risk
group has doubled.
The 61 patients who seem not to belong to any
of the recognized risk groups (tables 4-6) are
under investigation to determine whether new
population groups are at risk for AIDS. Unfor-
tunately, information regarding risk factors is in-
adequate for some of these patients because they
have died or cannot be interviewed at the time they
are reported. Another portion of the 61 patients
probably represents the expected "background"
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-
ever, be emerging from the study of these 61 pa-
tients. For example, five of the 61 patients are
women with PCP who deny iv drug abuse them-
selves but who have been steady sexual partners of
male iv drug abusers. One of these men had AIDS
himself. Five other patients with no apparent risk
factors were reported to have received a trans-
fusion of blood components within three years 'of
the onset of their illness. The donors of the blood
components received by these patients are now
under investigation.
Discussion
Because of the predominantly passive nature of
the CDC's present AIDS surveillance systems, the
results presented in this report must be interpreted
cautiously. Some AIDS cases may not be diag-
nosed, and others may be diagnosed but not re-
ported. These biases may vary over time and from
place to place. Active AIDS surveillance in
selected New York City hospitals and among pa-
tients with hemophilia is underway; programs are
being implemented by the CDC in collaboration
with the New York City Health Department and
the National Hemophilia Foundation.
Although the present surveillance has limita-
tions, it has established that AIDS is a rapidly
emerging, highly serious health problem in the
United States. Mortality has been very high.
Earlier diagnosis and treatment of KS and the op-
portunistic infections may improve survival. How-
ever, spontaneous return of normal immune func-
tion has not been reported in AIDS patients who
met the CDC case definition. Until a therapy to
reverse the immune dysfunction of AIDS becomes
available, mortality is likely to remain high.
The occurrence of AIDS among the diverse
population groups described in this report sug-
gests an infectious etiology. Reports of AIDS
among the sexual partners of homosexual and
heterosexual AIDS patients are consistent with
transmission of a putative "AIDS agent" by sexual
or other intimate person-to-person contact [11,
12]. Transmission of such an agent through blood
or blood products is consistent with the occur-
rence of AIDS in iv drug abusers, patients with
hemophilia, and transfusion recipients.
The overlap between the homosexual or bi-
sexual male risk group and the iv drug abuser
group might explain spread of an "AIDS agent"
from one group to the other. Geographically, this
overlap occurs particularly in New York City and
northern New Jersey.
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-
mented. Illnesses indicative of AIDS have oc-
curred in Haitians living in Haiti, and some
Haitians diagnosed in the United States became ill
before leaving Haiti [13] (AIDS Activity, CDC,
unpublished observations). Whether other Haitians
living in the United States acquired their disease
here or in Haiti is unknown.
Although a single-infectious-agent hypothesis
would seem to explain much of the AIDS epi-
demic, certain epidemiologic observations remain
puzzling. For example, what explains the excess of
KS cases among homosexual or bisexual men com-
pared with other AIDS risk groups? Perhaps the
route of transmission (sexual vs nonsexual) of an
"AIDS agent" into a susceptible host plays a role
in determining clinical outcome. Or perhaps a
cofactor present in the homosexual male popula-
tion, such as repeated exposure to cytomegalo-
virus or use of inhalant sexual stimulants, predis-
poses homosexual AIDS patients to develop KS.
The epidemiologic trends presented here suggest
that AIDS, a disease first recognized among
homosexual men in California and New York
City, is now becoming an increasing problem in
other population groups and in other parts of the
United States. Although these passive surveillance
data must be interpreted cautiously, the trends are
consistent with the gradual extension of an infec-
tious agent into new populations.
Although we believe that the diseases included
in the CDC surveillance definition are at least
moderately predictive of acquired cellular immune
deficiency, the spectrum of AIDS may not be
limited to KS and opportunistic infections. Other
unusual malignancies, including both a lymphoma
classified as either Burkitt's lymphoma or diffuse
undifferentiated non-Hodgkin's lymphoma [14,
15] and lymphoma limited to the brain [16], have
been reported among young homosexual men,
with or without previously described manifesta-
tions of AIDS. Autoimmune thrombocytopenia
associated with impaired cellular immunity has
been described among both homosexual men and
patients with hemophilia [17, 18]. Physicians from
several major metropolitan areas in the United
States have reported the occurrence of chronic,
generalized, unexplained lymphadenopathy in
homosexual men [19]. Many of these patients have
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
and a few have developed life-threatening mani-
festations of AIDS [20] (AIDS Activity, unpub-
lished observations). Finally, abnormalities of
cell-mediated immunity have been noted in both
asymptomatic homosexual men and patients with
hemophilia [21-25]. The meaning of this "asymp-
tomatic immunosuppression" is not clear. It may
represent a mild manifestation in the AIDS spec-
trum or may be an unrelated phenomenon.
Continuing surveillance will be needed to de-
termine whether AIDS will continue to increase in
incidence and appear in new population groups.
The full range of illness associated with AIDS may
not yet be known.
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