Systemic Sclerosis (Scleroderma)
CRITERIA FOR DIAGNOSIS*
Major
Thickening of the skin of the hands
Minor
Sclerodactyly (i.e., the changes of the major criterion but limited to
the fingers)
Digital pitting scars or loss of substance from the finger pad:
depressed areas at tips of fingers or loss of digital pad tissue as a
result of ischemia
Bibasilar pulmonary fibrosis
LUNG MANIFESTATIONS
Interstitial pulmonary fibrosis
Organizing pneumonia
Isolated pulmonary vascular disease
Aspiration pneumonia (secondary to esophageal dysmotility)
Chest wall restriction
*The major or ≥ 2 minor criteria required for diagnosis.
Rheumatoid Arthritis
CRITERIA FOR DIAGNOSIS*
Morning stiffness (lasting at least 1 hr)
Arthritis (soft tissue swelling or fluid) of 3 or more joints (PIP, MCP,
wrist, elbow, knee, ankle, MTP joints)
Arthritis of hand joints (swelling of at least 1 wrist, MCP, or PIP joint)
Symmetrical arthritis (i.e., simultaneous arthritis of the same joints on
both sides of the body)
Rheumatoid nodules
Serum rheumatoid factor positivity (at a level such that < 5% of
normal controls are positive)
Radiographic hand or wrist changes typical of rheumatoid arthritis
LUNG MANIFESTATIONS
Interstitial pulmonary fibrosis
Organizing pneumonia
Obliterative bronchiolitis
Follicular bronchiolitis
Bronchiectasis
Vasculitis
Nodules
Pleural disease
Lymphocytic interstitial pneumonia
Drug induced
*At least 4 criteria for a minimum of 6 weeks.
Systemic Lupus Erythematosus
CRITERIA FOR DIAGNOSIS*
Malar rash
Discoid rash
Photosensitivity skin rash
Oral or nasopharyngeal ulceration
Nonerosive arthritis involving ≥ 2 peripheral joints
Serositis (pleuritis or pericarditis)
Renal disorder (persistent proteinuria or cellular casts)
Neurologic disorder (unexplained seizures or psychosis)
Hematologic disorder (hemolytic anemia, leukopenia, lymphopenia,
or thrombocytopenia)
Immunologic disorder (positive LE cell, anti-DNA antibody, anti-Sm
antibody, false-positive syphilis serology)
Elevated antinuclear antibodies
LUNG MANIFESTATIONS
Acute lupus pneumonitis
Interstitial pulmonary fibrosis
Pulmonary vasculitis
Diffuse alveolar hemorrhage
Pulmonary hypertension
Shrinking lung syndrome
Antiphospholipid antibody syndrome
Organizing pneumonia
Pleural disease
*Minimum of 4 criteria required.
Polymyositis with Dermatomyositis
CRITERIA FOR DIAGNOSIS
Symmetrical proximal muscle weakness
Muscle biopsy specimen showing myositis
Elevation of serum skeletal muscle enzymes
Characteristic electromyographic pattern of myositis
Typical rash of dermatomyositis
LUNG MANIFESTATIONS
Interstitial pulmonary fibrosis
Acute pneumonitis (with diffuse alveolar damage)
Organizing pneumonia
Aspiration pneumonia
Pulmonary vasculitis and alveolar hemorrhage
Respiratory muscle weakness
Behçet Syndrome
CRITERIA FOR DIAGNOSIS
Major (required)
Recurrent aphthous ulceration at least 3 times in a 12-mo period
Minor (2 of 4)
Recurrent genital ulceration
Ocular disease
Skin lesions (erythema nodosum, skin ulcers)
Positive pathergy test (a 2-mm erythematous papule or pustule at
the prick site 48 hr after the application of a sterile hypodermic
20- to 22-gauge needle that obliquely penetrated avascular
antecubital skin to a depth of 5 mm)
