ARA Nomenclature and Classification of Arthritis and Rheumatism (Tentative)

By BARUCH S. BLUMBERG, CHAIRMAN, JOSEPH J. BUNIM, EVAN CALMNS, CONRAD L. PIRANI AND NATHAN J. ZVAIFLER~

N 1960 the editor of ARTHENTIS AND RHEUMATISM requested that the presi- I dent of the American Rheumatism Association appoint a committee to prepare a nomenclature of arthritis and rheumatism. The committee was ap- pointed and, in June, 1961, reported that it could not provide a nomenclature without an acceptable classification. I t noted that the ARA had no official nomenclature and classification with the possible exception of the table “The Classification of Rheumatic Diseases” which appeared in the Primer on Rheu- nmtic Diseases. There had been no major revision of this list for approxi- mately 20 years. Other classifications which were available, but had not been officially accepted by the ARA, were included in the “International Classifica- tion of Disease” of the World Health Organization, “The Standard Nomencla- ture of Disease,” and the “Nomenclature and Classification of Arthritides and Other Rheumatic Disorders.” The last was tentatively adopted by the Inter- national League Against Rheumatism at Toronto in 1957.

A nomenclature and classification committee was appointed, and its sug- gested list with minor changes, was accepted by the President and Executive Committee of the ARA on December 5, 1963. The present paper contains an introductory section extracted from the December 5 report of the committee and the ARA Nomenclature and Classification of Arthritis and Rheumatism ( Tentative).

Nomenclature is defined as “a system or set of names or designations used in a particular science, discipline or art and formally adopted or sanctioned by the usage of its practitioners.” CIassification is defined as “the act, or a method of classifying” and “classify” is “to group or segregate in classes that have systemic relations usually founded on common properties or characters.” The disease entities and syndromes considered under the heading of “arthritis and rheumatism” have one feature in common, namely, that they include symptoms or findings referable to the joints or adjacent tissues at some time during the illness. This results in a heterogeneous group of diseases which may not have many other features in common and, therefore, classification is difficult and arbitrary. An etiological classification would be satisfactory, but the etiologies of most of the entities are unknown, and a classification based on misconcep- tion is worse than none at all. A simple listing of the diseases with no attempt at classification also has disadvantages in that it makes the list cumbersome and dull for use by students and others.

This nomenclature and classification is not exhaustive, but, we hope) in-

*The Nomenclature and Classification Committee of the American Rheumatism Associa- tion.

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ARTHRITIS AND RHEUMATISM, Vor.. 7, No. 1 (FEBRUARY), 1964

94 BLUMBERG ET AL.

chides most of the diagnosis used in clinical practice. The objective of the classification was to arrange the entities in some convenient form, without, at the same time making unsound commitments as to common etiology or general similarity between the diseases in a particular category. One of the great dangers of an official classification is that it solidifies thinking. The present list, therefore, should be considered as tentative and subject to change when required.

We are indebted to Drs. Boland and Steinbrocker who served as consultants to the committee.

Nomenclature and Classification (Tentative)

I. Polyurthritis of unknown etiology A. Rheumatoid arthritis B. Juvenile rheumatoid arthritis ( Still’s disease) C. Ankylosing spondylitis D. Psoriatic arthritis E. Reiter’s syndrome F. Other

11. “Connectiue Tissue” disorders A. Systemic lupus erythematosus B. Periarteritis nodosa C. Scleroderma (Progressive systemic sclerosis ) D. Polymyositis and dermatomyositis E. Other

111. Hheuwutic fever

IV. Degenerutiue joint diseuse (osteonrthritis, osteourthrosis) A. Primary B. Secondary

V. Non-articulur rheumatism A. Fibrositis B. Intervertebral disc and low back syndromes C. Myositis and myalgia D. Tendinitis and peritendinitis (bursitis) E. Tenosynovitis F. Fasciitis G. Carpal tunnel syndrome H. Other

(See also shoulder-hand syndrome, VIII E)

VI. Diseuses with which arthritis is frequently associated A. Sarcoidosis

NOhIENCLATUHE AND CLASSIFICATION 95

B. Relapsing polychondritis C. Henoch-Schonlein syndrome D. Ulcerative colitis E. Regional ileitis F. Whipple’s disease G. Sjogren’s syndrome H. Familial Mediterranean Fever I. Other

(See also psoriatic arthritis, I D )

VII. Associated with known infectious agents A. Bacterial

1. Brucella 2. Gonococcus 3. Mycobacterium tuberculosis 4. Pneumococcus 5. Salmonella 6. Staphylococcus 7. Streptobacillus moniliformis ( Haverhill fever ) 8. Treponema pallidum ( syphilis ) 9. Treponema pertenue (yaws)

10. Others B. Rickettsia1 C. Viral D. Fungal E. Parasitic

(See also rheumatic fever, 111)

VIII. Truunuztic untE/or neurogenic disorders A. Traumatic arthritis (i.e., the result of direct trauma) B. Lues (tertiary syphilis) C. Diabetes D. Syringomyelia E. Shoulder-hand syndrome F. Mechanical derangements of joints G. Others

(See also, Degenerative joint disease, IV; Carpal tunnel syndrome, V. G )

IX. Associated w4h known biochemical m endocrine abnormalities A. Gout B. Ochronosis C. Hemophilia D. Hemoglobinopathies ( e.g., Sickle cell disease) E. Agammaglobulinemia F. Gaucher’s disease

96 BLUMBERG EX AL.

G. Hyperparathyroidism H. Acromegaly I. Hypothyroidism J. Scurvy (Hypovitaminosis C)

K. Xanthoma tuberosum L. Other

(See also, Multiple myeloma, X74; Hurler’s syndrome, XI1 C) .

X. Tumors and tumor-like conditions A. Synovioma B. Pigmented villonodular synovitis C. Giant cell tumor of tendon sheath D. Primary juxta-articular bone tumors E. Metastatic F. Leukemia G. Multiple myeloma H. Benign tumors of articular tissue I. Others

( See also hypertrophic osteoarthropathy XIII-G )

XI. Allergy and drug reuctions A. Arthritis due to specific allergens (viz., serum sickness) B. Arthritis due to drugs (viz., hydralazine syndrome) C. Other

XII. Inherited and congenital disorders A. Marfan’s syndrome B. Ehlers-Danlos syndrome C. Hurler’s syndrome D. Congenital hip dysplasia E. Morquio’s disease F. Other

XIII. hliscelluneous disorders A. Amyloidosis B. Aseptic necrosis of bone C . Behcet’s syndrome D. Chondrocalcinosis (pseudo-gout ) E. Erythema multiforme ( Stevens-Johnson syndrome ) F. Erythema nodosum G. Hypertrophic osteoarthropathy H. Juvenile osteochondritis I. Osteochondritis dissecans J. Reticulohistiocytosis of joints ( lipoid dermato-arthritis ) K. Tietze’s disease L. Other

NOMENCLATURE AND CLASSIFICATION 97

Baruch S . Blumberg, M.D., Ph.D., Chief, Program of Geo- graphic Medicine and Genetics, National Institute of Arthritis and Metabolic Diseases, National Institutes of Health, Bethes-

da, Md.

Joseph J. Bunim, M.D., Clinical Director, National Institute of Arthritis and Metabolic Dismsc?s, National Institutes of Health, Bethesdu, Maryland; Associate Professor of Medicine, Johns Hopkins University, Baltimore, Maryland; Clinical Pro- fessor of Medicine, Georgetown University School of Medicine,

Washington, D. C.

Evan Calkins, M.D., Professor of Medicine, State University of N e w York at Bufalo; Head, Dqartment of Medicine,

BLcfalo General HosT)ital

Conrad L. Pirani, M.D., Professor of Pathology, University of Illinois College of Medicine, Chicago, Il l .

Nathan J. Zvaifler, M.D., Assistant Professor of Medicine, Gemgetown University School of Medicine, Washington, D. C.