APPENDIX I Criteria for the Classifi cation and Diagnosis of the Rheumatic Diseases The criteria presented in the following section have The proposed criteria are empiric and not intended been developed with several different purposes in mind. to include or exclude a particular diagnosis in any indi- For a given disorder, one may have criteria for (1) clas- vidual patient. They are valuable in offering a standard sifi cation of groups of patients (e.g., for population to permit comparison of groups of patients from differ- surveys, selection of patients for therapeutic trials, or ent centers that take part in various clinical investiga- analysis of results on interinstitutional patient compari- tions, including therapeutic trials. sons); (2) diagnosis of individual patients; and (3) esti- The ideal criterion is absolutely sensitive (i.e., all mations of disease frequency, severity, and outcome. patients with the disorder show this physical fi nding or The original intention was to propose criteria as the positive laboratory test) and absolutely specifi c guidelines for classifi cation of disease syndromes for the (i.e., the positive fi nding or test is never present in any purpose of assuring correctness of diagnosis in patients other disease). Unfortunately, few such criteria or sets taking part in clinical investigation rather than for indi- of criteria exist. Usually, the greater the sensitivity of a vidual patient diagnosis. However, the proposed criteria fi nding, the lower its specifi city, and vice versa. When have in fact been used as guidelines for patient diagno- criteria are established attempts are made to select rea- sis as well as for research classifi cation. One must be sonable combinations of sensitivity and specifi city. cautious in such application because the various criteria An updated listing of additional criteria sets for rheu- are derived from the use of analytic techniques that matic and musculoskeletal disorders is available on the allow the minimum number of variables to achieve the American College of Rheumatology website (http:// best group discrimination, rather than to attempt to www.rheumatology.org/publications/classification/ arrive at a diagnosis in an individual patient. index.asp?aud=mem). CRITERIA FOR THE CLASSIFICATION Of FIBROMYALGIAa 1. History of widespread pain Defi nition. Pain is considered widespread when all of the following are present: pain in the left side of the body, pain in the right side of the body, pain above the waist, and pain below the waist. In addition, axial skeletal pain (cervical spine or anterior chest or thoracic spine or low back) must be present. In this defi nition, shoulder and buttock pain is considered as pain for each involved side. “Low back” pain is considered lower segment pain. 2. Pain in 11 of 18 tender point sites on digital palpation.b Defi nition. Pain, on digital palpation, must be present in at least 11 of the following 18 tender point sites: Occiput: bilateral, at the suboccipital muscle insertions. Low cervical: bilateral, at the anterior aspects of the intertransverse spaces at C5–C7. Trapezius: bilateral, at the midpoint of the upper border. Supraspinatus: bilateral, at origins, above the scapula spine near the medial border. Second rib: bilateral, at the second costochondral junctions, just lateral to the junctions on upper surfaces. Lateral epicondyle: bilateral, 2 cm distal to the epicondyles. Gluteal: bilateral, in upper outer quadrants of buttocks in anterior fold of muscle. Greater trochanter: bilateral, posterior to the trochanteric prominence. Knee: bilateral, at the medial fat pad proximal to the joint line. SOURCE: Adapted from Wolfe F, Smythe HA, Yunus MS, et al. The American College of Rheumatology 1990 criteria for the classifi cation of fi bromyalgia. Report of the multicenter criteria committee. Arthritis Rheum 1990;33:100–172, with permission of the American College of Rheumatology. a For classifi cation purposes, patients will be said to have fi bromyalgia if both criteria are satisfi ed. Widespread pain must have been present at least 3 months. The presence of a second clinical disorder does not exclude the diagnosis of fi bromyalgia. b Digital palpation should be performed with an approximate force of 4 kg. For a tender point to be considered “positive” the subject must state that the palpa- tion was painful. “Tender” is not to be considered “painful.” 669 670 APPENDIX I CRITERIA FOR THE CLASSIFICATION OF RHEUMATOID ARTHRITISa CRITERION DEFINITION 1. Morning stiffness Morning stiffness in and around the joints, lasting at least 1 hour before maximal improvement 2. Arthritis of three or more joint areas At least three joint areas simultaneously have had soft tissue swelling or fl uid (not bony overgrowth alone) observed by a physician. The 14 possible areas are right or left PIP, MCP, wrist, elbow, knee, ankle, and MTP joints 3. Arthritis of hand joints At least one area swollen (as defi ned above) in a wrist, MCP, or PIP joint 4. Symmetric arthritis Simultaneous involvement of the same joint areas (as defi ned in 2) on both sides of the body (bilateral involvement of PIPs, MCPs, or MTPs is acceptable without absolute symmetry) 5. Rheumatoid nodules Subcutaneous nodules, over bony prominences, or extensor surfaces, or in juxta-articular regions, observed by a physician 6. Serum rheumatoid factor Demonstration of abnormal amounts of serum rheumatoid factor by any method for which the result has been positive in ≤ 5% of normal control subjects 7. Radiographic changes Radiographic changes typical of rheumatoid arthritis on posteroanterior hand and wrist radiographs, which must include erosions or unequivocal bony decalcifi cation localized in or most marked adjacent to the involved joints (osteoarthritis changes alone do not qualify) SOURCE: Reprinted from Arnett FC, Edworthy SM, Bloch DA. et al. The American Rheumatism Association 1987 revised criteria for the classifi cation of rheuma- toid arthritis. Arthritis Rheum 1988;31:315–324, with permission of the American College of Rheumatology. ABBREVIATIONS: MCP, metacarpophalangeal; MTP, metatarsophalangeal; PIP, proxomal interphalangeal. a For classifi cation purposes, a patient shall be said to have rheumatoid arthritis if he/she has satisfi ed at least four of these seven criteria. Criteria 1 through 4 must have been present for at least 6 weeks. Patients with two clinical diagnoses are not excluded. Designation as classic, defi nite, or probable rheumatoid arthritis is not to be made. CLASSIFICATION OF PROGRESSION OF RHEUMATOID ARTHRITIS Stage I, Early *1. No destructive changes on roentgenographic examination 2. Radiographic evidence of osteoporosis may be present Stage II, Moderate *1. Radiographic evidence of osteoporosis, with or without slight subchondral bone destruction; slight cartilage destruction may be present *2. No joint deformities, although limitation of joint mobility may be present 3. Adjacent muscle atrophy 4. Extra-articular soft tissue lesions, such as nodules and tenosynovitis may be present Stage III, Severe *1. Radiographic evidence of cartilage and bone destruction, in addition to osteoporosis *2. Joint deformity, such as subluxation, ulnar deviation, or hyperextension, without fi brous or bony ankylosis 3. Extensive muscle atrophy 4. Extra-articular soft tissue lesions, such as nodules and tenosynovitis may be present Stage IV, Terminal *1. Fibrous or bony ankylosis 2. Criteria of stage III SOURCE: Reprinted from Steinbrocker O, Traeger CH. Batterman RC. Therapeutic criteria in rheumatoid arthritis. JAMA 1949;140:659–662, with permission. * The criteria prefaced by an asterisk are those that must be present to permit classifi cation of a patient in any particular stage or grade. CRITERIA FOR THE CLASSIFICATION AND DIAGNOSIS 671 CRITERIA FOR CLINICAL REMISSION IN RHEUMATOID ARTHRITISa Five or more of the following requirements must be fulfi lled for at least 2 consecutive months: 1. Duration of morning stiffness not exceeding 15 minutes 2. No fatigue 3. No joint pain (by history) 4. No joint tenderness or pain on motion 5. No soft tissue swelling in joints or tendon sheaths 6. Erythrocyte sedimentation rate (Westergren method) less than 30 mm/hour for a female or 20 mm/hour for a male SOURCE: Reprinted from Pinals RS, Masi AT, Larsen RA. et al. Preliminary criteria for clinical remission in rheumatoid arthritis. Arthritis Rheum 1981;24:1308– 1315, with permission of the American College of Rheumatology. a These criteria are intended to describe either spontaneous remission or a state of drug-induced disease suppression, which simulates spontaneous remission. No alternative explanation may be invoked to account for the failure to meet a particular requirement. For instance, in the presence of knee pain, which might be related to degenerative arthritis, a point for “no joint pain” may not be awarded. Exclusions: Clinical manifestations of active vasculitis, pericarditis, pleuritis or myositis, and unexplained recent weight loss or fever attributable to rheumatoid arthritis will prohibit a designation of complete clinical remission. CRITERIA FOR CLASSIFICATION OF FUNCTIONAL STATUS IN RHEUMATOID ARTHRITISa Class I Completely able to perform usual activities of daily living (self-care, vocational, and avocational) Class II Able to perform usual self-care and vocational activities, but limited in avocational activities Class III Able to perform usual self-care activities, but limited in vocational and avocational activities Class IV Limited in ability to perform usual self-care,