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Human immunodeficiency virus (HIV) infection codes and new codes for Kaposi's sarcoma.


These addenda for Volumes 1 and 2 of the International Classification of Diseases, 9th Revision, Clinical Modification (ICD-9-CM), are reported by the World Health Organization Collaborating Center for Classification of Diseases for North America at the National Center for Health Statistics.

These addenda replace the addendum containing codes for human immunodeficiency virus (HIV) infection (042.0-044.9) that were effective January 1, 1988. These addenda will be effective October 1, 1991, and are the second revision of these codes for the classification of HIV infection. These addenda incorporate minor changes in content of the classification reflecting new scientific knowledge. The structure of the classification, the codes within the classification, and the manner in which the codes may be used remain unchanged. These changes are effective only for morbidity purposes; the cause-of-death codes are unchanged.

Within these revised addenda are three basic modifications:

1. The secondary codes for Kaposi's sarcoma have been changed to reflect the creation of a new three-digit category for this condition (176).

2. Several new clinical conditions have been added to the "Includes only" notes:

-- Acute or subacute endocarditis (421.0,421.9), microsporidiosis (136.8), acute or subacute myocarditis (422.90, 422.99), other bacterial pneumonia (482.0-482.9) and pneumococcal pneumonia (481) under 042.1

-- Histiocytic or large cell lymphoma (200.0) under 042.2

-- Secondary cardiomyopathy (425.9) and nephritis and nephropathy in conditions classified elsewhere (580-583) under 043.3.

3. Several categories of HIV manifestations have been expanded to include other, similar conditions:

-- Viral pneumonia NEC and NOS (480.8 and 480.9) under 042.0

-- Encephalitis (323.8, 323.9), encephalomyelitis (323.8 and 323.9), and myelitis (323.8 and 323.9) under 043.1.


The increasing incidence of HIV infection and advances in medical knowledge about the spectrum of illness caused by this virus have created demand for continued modifications to the classification. These modifications will improve the accuracy of reporting and allow public health officials, clinical researchers, and agencies that finance health care to monitor diagnoses of acquired immunodeficiency syndrome (AIDS) and other manifestations of HIV infection.

When the original interim classification was issued on October 1, 1986, it was anticipated that periodic revisions would be required. The first such revision occurred in 1988. It characterized the causative agent and reflected the change in terminology from human T-cell lymphotropic virus-lll/lymphadenopathy-associated virus (HTLV-lll/LAV) to HIV. The 1988 revision reflected the state of medical knowledge at that time, but additional manifestations have since been found to be associated with HIV infection.


The classification consists of three 3-digit rubrics with 4-digit subdivisions utilizing the ICD-9-CM codes 042, 043, 044 in Chapter 1, Infectious and Parasitic Diseases. This places HIV infection at the beginning of viral diseases. The categories are defined primarily by the manifestations of the infection. Clear identification of the manifestations s is required for the selection of the correct code. Multiple coding of all listed manifestations of HIV infection is required. Furthermore, it is also necessary that certain of the manifestations be listed in a "due to" or other causal relationship in order to select the appropriate code. Other manifestations need only be listed on the record with the HIV infection to assume a "with" relationship. Because of the difficulties inherent in this method of arriving at the correct code, this document includes an alphabetical table of manifestations in the "with" and "due to" relationships with HIV infections and the appropriate code. Coders are instructed to always use the table first as they would the index of ICD-9-CM.


This classification is not intended for purposes of staging or specifying severity of illness. Rather, it is based on well-defined groupings of disease manifestations most compatible with the manner in which patients with HIV infection are currently categorized by providers of health-care services, clinical investigators, researchers, and public health officials. Thus, the spectrum of HIV infection is divided into three categories.

1. HIV infection with specified secondary infections or malignant neoplasms, or AIDS (042)

2. HIV infection with other specified manifestations in the absence of either specified secondary infections or malignant neoplasms (043)

3. Other HIV infection not classifiable above (044).


The use of unacceptable terminology and abbreviations should be discouraged. AIDS is not synonymous with HIV infection or with such terms as pre-AIDS and AIDS-related complex (ARC) or syndromes.


To use these codes correctly, the physician must provide complete information and state the relationship between HIV infection and other conditions. It will not be unusual for a patient suffering from HIV infection to be admitted for an unrelated condition.

The classification requires that the relationship between HIV infection, the manifestations, and other listed conditions be identified. The term "with" implies that the condition or manifestation of HIV infection need only be listed on the record. Terms such as "and" and "in association with" will be considered in the same manner as "with."

The term "due to" is used in this classification to denote a causal relationship. The broad definition of "due to" is implied here as it is used in the International Classification of Diseases, 9th Revision.

The words "due to (or as a consequence of)" that appear on the form of medical certificate include not only etiological or pathological sequences, but also sequences where there is no such direct causation but where an antecedent condition is believed to have prepared the way for the direct cause by damage to tissues or impairment of function even after a long interval [1].

All manifestations of HIV infection must be coded. The alphabetical table will help the coder select the most appropriate code for the HIV infection in association with the most common manifestations. Other manifestations not found on the table may be reported; the selection of the appropriate 042-044 code is determined solely by the terminology used for the HIV infection. Codes 042, 043, and 044 are mutually exclusive and should never be listed together on the same record. Priority is given to 042 over 043 and 044; 043 is given priority over 044.

Only one code from the 042-044 series should be used. For instance, a patient with candidiasis of the lung (112.4), Kaposi's sarcoma (176.0-176.9), and HIV infection described as AIDS would be assigned only one 042 code. The coder should select a single HIV code based on the decision of the attending physician as to the most descriptive code for the admission. This code may change during subsequent admissions.

If a person has ever been diagnosed as having AIDS or any of the HIV manifestations classifiable to 042, a code from 042 should be assigned for all subsequent admissions even if the patient is currently under treatment for a condition that would otherwise be assignable to 043 or 044. Similarly, if a patient has ever been diagnosed with ARC or any condition classifiable to 043, a code from 044 should not be used. In these instances, a fourth digit of .9 ("unspecified") should be used with the HIV infection code.

In morbidity use, selection of the principal diagnosis should be based upon the information contained in the individual record of the patient's hospitalization. Selection of the principal diagnosis and additional diagnoses applies only to hospitalized patients. The HIV infection codes can be used as either the principal or additional diagnosis depending upon the circumstances of the admission. The notes "with" and "due to HIV infection" do not imply a particular sequence.

This classification system will be used while additional scientific information on the pathogenesis and natural history of HIV infection accumulates. This revision follows 3 years of use. This system will continue to be reviewed for its appropriateness in classifying HIV infection and its disease manifestations.


[1.] World Health Organization. Manual of the international statistical classification of diseases, injuries, and causes of death, based on the recommendations of the Ninth Revision Conference, 1975. Geneva: World Health Organization, 1977:700.

Human Immunodeficiency Virus (HIV) Infection (042-044)

Note: In this classification, the following terms are used to define and to represent other terms that are referable to these categories 042-044.

1. AIDS: Acquired immune deficiency syndrome Acquired immunodeficiency syndrome

2. AIDS-like syndrome:

AIDS-like disease (illness) (syndrome) AIDS-related complex AIDS-related conditions ARC Pre-AIDS Prodromal-AIDS

3. HIV-infection (disease) (illness):

AAV (disease) (illness) (infection) AIDS-associated retrovirus (disease) (illness) (infection) AIDS-associated virus (disease) (illness) (infection) AIDS-related virus (disease) (illness) (infection) AIDS virus (disease) (illness) (infection) ARV (disease) (illness) (infection) Human immunodeficiency virus (disease) (illness) (infection) Human immunovirus (disease) (illness) (infection) Human T-cell lymphotropic virus-III (disease) (illness) (infection) HTLV-III (disease) (infection) HTLV-III/LAV (disease) (illness) (infection) LAV (disease) (illness) (infection) LAV/HTLV-III (disease) (illness) (infection) Lymphadenopathy-associated virus (disease) (illness) (infection)

042 Human immunodeficiency virus infection with specified conditions

Includes: acquired immunodeficiency syndrome (AIDS)

042.0 With specified infections

Includes only: candidiasis of lung (112.4) coccidiosis (007.2) cryptosporidiosis (007.2) isosporiasis (007.2 cryptococcosis (117.5) With HIV infection pneumocystosis (136.3) progressive multifocal leukoencephalopathy (046.3) toxoplasmosis (130)

042.1 Causing other specified infections:

Includes only: candidiasis disseminated (112.5) of: mouth (112.0) skin and nails (112.3) other and unspecified sites (112.8, 112.9) (excludes: 112.1, 112.2, 112.4) coccidioidomycosis (114) cytomegalic inclusion disease (078.5) endocarditis, acute or subacute (421.0, 421.9) herpes simplex (054) herpes zoster (053) histoplasmosis (115) microsporidiosis (136.8) mycobacteriosis, other and unspecified (031.8, 031.9) Due to HIV infection (excludes: 031.0, 031.1) myocarditis, acute or subacute (422.90, 422.99) Nocardia infection (039) opportunistic mycoses (118) pneumonia: NOS (486) other bacterial (482.0-482.9) pneumococcal (481) viral NEC and NOS (480.8, 480.9) Salmonella infections (003.1-003.9) (excludes: gastroenteritis 003.0) septicemia (038) strongyloidiasis (127.2) tuberculosis (010-018)

042.2 With specified malignant neoplasms:

Includes only: Burkitt's tumor or lymphoma (200.2) immunoblastic sarcoma (200.8) Kaposi's sarcoma (176.0-176.9) lymphoma: With HIV infection histiocytic (200.0) large cell (200.0) primary lymphoma of the brain (202.8) reticulosarcoma (200.0)

042.9 Acquired immunodeficiency syndrome, unspecified

AIDS with other conditions classifiable elsewhere except as in 042.0-042.2

043 Human immunodeficiency virus infection causing other specified conditions

Includes: AIDS-like syndrome AIDS-related complex ARC Excludes: HIV infection classifiable to 042

043.0 Causing lymphadenopathy

Enlarged lymph nodes (785.6) Due to HIV infection Swollen glands (785.6)

043.1 Causing specified diseases of the central nervous system

Includes only: central nervous system: demyelinating disease NOS (341.9) disorders NOS (348.9, 349.9) non-arthropod-borne viral diseases, other and unspecified (049.8, 049.9) slow virus infection, other and unspecified (046.8, 046.9) dementia: NOS (298.9) Due to HIV infection organic (294.9) presenile (290.1) encephalitis (323.8, 323.9) encephalomyelitis (323.8, 323.9) encephalopathy (348.3) myelitis (323.8, 323.9) myelopathy (336.9) organic brain syndrome NOS (nonpsychotic) (310.9) psychotic (294.9)

043.2 Causing other disorders involving the immune mechanism

043.3 Causing other specified conditions

Includes only: abnormal weight loss (783.2) abnormality, respiratory (786.0) agranulocytosis (288.0) anemia: NOS (285.9) aplastic, other and unspecified (284.8, 284.9) Due to HIV infection deficiency (280-281) hemolytic, acquired (283) arthritis: pyogenic (711.0) infective (711.9) blindness or low vision (369) blood and blood-forming organs, unspecified disease (289.9) cachexia (799.4) cardiomyopathy, secondary (425.9) dermatomycosis (111) dermatophytosis (110) diarrhea (noninfectious) (588) infectious (009) disease or disorder NOS: blood and blood-forming organs (289.9) salivary gland (527.9) skin and subcutaneous tissue (709.9) dyspnea (786.0) fatigue (780.7) fever (780.6) gastroenteritis (noninfectious) (558) infectious (009) hepatomegaly (789.1) hyperhidrosis (780.8) hypersplenism (289.4) infection: intestinal, ill-defined (009) Due to HIV infection lack of expected physiological development in infant (783.4) leukoplakia of oral mucosa (tongue) (528.6) malabsorption, intestinal (579.9) malaise (780.7) nephritis and nephropathy (580-583) neuralgia NOS (729.2) neuritis NOS (729.2) nutritional deficiencies (260-269) pneumonitis, lymphoid, interstitial (516.8) polyneuropathy (357.0, 357.8, 357.9) pyrexia (780.6) radiculitis NOS (729.2) rash NOS (782.1) retinal vascular changes (362.1) retinopathy, background (362.1) splenomegaly (789.2) thrombocytopenia, secondary and unspecified (287.4, 287.5) volume depletion (276.5)

043.9 Acquired immunodeficiency syndrome-related complex, unspecified

AIDS-related complex (ARC) with other conditions classifiable elsewhere except as in 042.0-043.3

044 Other human immunodeficiency virus infection

Excludes: HIV infection classifiable to 042 or 043

044.0 Causing specified acute infections

Includes only:

acute lymphadenitis (683)

aseptic meningitis (047.9) Due to HIV infection viral infection ("infectious mononucleosis-like syndrome") (079.9)

444.9 Human immunodeficiency virus infection, unspecified

HIV infection with other conditions classifiable elsewhere except as in 042.0-044.0

795.8 Positive serological or viral culture findings for human immunodeficiency virus (HIV)

044 Other human immunodeficiency virus infection

Excludes: HIV infection classifiable to 042 or 043

044.0 Causing specified acute infections

Includes only:

acute lymphadenitis (683)

aseptic meningitis (047.9) Due to HIV infection viral infection ("infectious mononucleosis-like syndrome") (079.9)

444.9 Human immunodeficiency virus infection, unspecified

HIV infection with other conditions classifiable elsewhere except as in 042.0-044.0

795.8 Positive serological or viral culture findings for human immunodeficiency virus (HIV)
               Other HIV Statements
Carrier (of)
  AIDS virus                                VO2.9
  HIV                                       VO2.9
Contact (with)
  AIDS virus                                VO1.8
  HIV                                       VO1.8
Exposure (to)
  AIDS virus                                VO1.8
  HIV                                       VO1.8
  culture (for)
    AIDS virus                              795.8
    HIV                                     795.8
  serology (for)
    AIDS virus                              795.8
    HIV                                     795.8

New ICD-9-CM Codes for Kaposi's Sarcoma

The International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) previously classified Kaposi's sarcoma under category 173, "Other malignant neoplasm of skin," regardless of the site of involvement. However, Kaposi's sarcoma is being reported in many sites other than skin, most notably in the gastrointestinal tract and lung, and at times in other sites including the brain. There is no way under the previous scheme to identify the site of involvement. Coding practices for Kaposi's sarcoma have been inconsistent as coders struggled to find methods within the previous classification to allow for site identification. The revised classification of Kaposi's sarcoma, effective October 1, 1991, is as follows:

176 Kaposi's Sarcoma

176.0 Skin

176.1 Soft tissue Includes: blood vessel ligament connective tissue lymphatic(s) NEC fascia muscle

Excludes: lymph glands and nodes (176.5)

176.2 Palate

176.3 Gastronintestinal sites

176.4 Lung

176.5 Lymph nodes

176.8 Other specified sites Includes: oral cavity NEC

176.9 Unspecified Viscera NOS
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Publication:Morbidity and Mortality Weekly Report
Date:Jul 26, 1991
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