ARA Scleroderma Criteria Cooperative Study Preliminary Clinical Criteria for Systemic Sclerosis

Clinical terms for changes seen in patients with systemic sclerosis (Table 2, page 583)

(1) typical sclerodermatous skin changes

(1a) changes: tightness, thickening, non-pitting induration

(1b) exclusions: localized scleroderma (morphea, linear scleroderma)

(1c) sclerodactyly: changes limited to fingers and/or toes

(1d) proximal scleroderma: changes proximal to the metacarpophalangeal or metatarsophalangeal joints; involving other parts of the extremities, face, neck or trunk; involvement usually bilateral and symmetrical; almost always associated with sclerodactyly

(2) other skin changes:

(2a) digital pitting scars: depressed areas at the tips of digits as a result of digital ischemia rather than trauma or exogenous causes

(2b) loss of substance from the finger pad: loss of digital pad tissue as a result of digital ischemia rather than trauma or exogenous causes

(2c) bilateral finger or hand edema: firm but pitting edema; fingers may show a puffy, sausage-like swelling; hand edema often involves the dorsal hand

(2d) abnormal skin pigmentation: hyperpigmentation often containing areas of punctate or patchy hypopigmentation or depigmentation ("pepper and salt")

(2e) telangiectasia: visible macular dilatation of superficial blood vessels which collapse upon pressure and fill slowly when pressure is released; differential diagnosis includes spider angiomas (rapidly filling) and dilated linear superficial vessels

(2f) Raynaud's phenomenon: at least a 2-phase color change in the fingers and often toes, consisting of pallor, cyanosis, and/or reactive hyperemia in response to cold exposure or emotion

(3) visceral manifestations:

(3a) bibasilar pulmonary fibrosis: bilateral reticular pattern of linear or lineonodular densities which are most pronounced in basilar portions of the lungs on standard chest X-rays; it may progress to diffuse mottling or "honeycomb lung"; changes should not be attributable to primary lung disease

(3b) distal esophageal dysphagia: substernal discomfort on swallowing or sensation of food holdup in the retrosternal location

(3c) distal esophageal dysmotility: hypoperistalsis or aperistalsis, often accompanied by evidence of a decrease in the lower esophageal sphincter tone with reflux

(3d) colonic sacculations: wide-mouthed diverticula of colon located along the antimesenteric border; may also occur in the ileum and jejunum

Major criteria for diagnosis (Table 9, page 587):

(1) proximal scleroderma

Minor criteria for diagnosis (Table 9, page 587):

(1) sclerodactyly

(2) digital pitting scars of fingertips or loss of substance of the distal finger pad (? also include toes)

(3) bibasilar pulmonary fibrosis

Criteria for the diagnosis of systemic sclerosis - one or both of the following"

(1) 1 major criteria

(2) 2 or 3 minor criteria

Performance:

• The major criteria alone: 91% sensitive, 99+% specific.

• The criteria identified 97% of definite systemic sclerosis patients, but only 2% of comparison population (SLE, polymyositis/dermatomyositis, Raynaud's phenomenon).