Furst et al developed indices of organ involvement for patients with systemic sclerosis. The scores can be used at initial assessment and for monitoring the patient over time. Changes in the indices can help determine the effectiveness of therapeutic interventions.
Organ indices (10):
(1) pulmonary
(2) skeletal
(3) cutaneous
(4) upper gastrointestinal
(5) lower gastrointestinal
(6) muscle
(7) cardiac
(8) right heart
(9) left heart
(10) renal
|
Pulmonary |
Finding |
Points |
|---|---|---|
|
shortness of breath |
absent |
0 |
|
|
present |
3 |
|
pulmonary fibrosis |
absent |
0 |
|
|
present |
6 |
|
carbon monoxide diffusing capacity (DLco), percent of normal, as a decimal |
|
2 / (decimal) |
maximum pulmonary score = none
|
Skeletal |
Finding |
Points |
|---|---|---|
|
joint tenderness count |
(joints tender to palpation) |
0 - 29 |
|
radiographic evidence of joint disease |
absent |
0 |
|
|
present |
5 |
|
hand or joint radiograph with changes of systemic sclerosis, with subcutaneous calcinosis and/or acro-osteolysis |
absent |
0 |
|
|
present |
5 |
where:
• The joint tenderness count is the number of joints tender to palpation. Minimal score = 0; maximal score 29).
• To get a maximal score of 39, the hand or joint radiographs must contribute a maximum of 5 points. To get this, there are 2 possible options. (1) Give 2.5 points if changes in hand and 2.5 points if changes in another joint; (2) Multiple 2.5 by 2 if present. I have chosen option (2); the table shows a value of +2.5 in the assigned value and in the text says "0 = no, 2 = present".
maximum skeletal score = 39
|
Cutaneous |
Finding |
Points |
|---|---|---|
|
sclerodactyly |
absent |
0 |
|
|
present |
4 |
|
subcutaneous calcinosis |
absent |
0 |
|
|
present |
2 |
|
ulcers of skin and/or fingers |
absent |
0 |
|
|
present |
2 |
|
skin score of Furst et al (hidebinding or tethering) |
|
0 - 30 |
where:
• The hidebinding/tethering score is measured at 10 sites, each graded 0 –3, for a maximal score of 30. The score is described previously (above).
maximum cutaneous score = 38
|
Upper Gastrointestinal |
Finding |
Points |
|---|---|---|
|
typical systemic sclerosis changes on cine-esophagram, manometry or on endoscopy |
absent |
0 |
|
|
present |
2 |
|
esophageal stricture |
absent |
0 |
|
|
present |
2 |
|
dysphagia |
absent |
0 |
|
|
present |
2 |
maximum upper GI score = 6
|
Lower Gastrointestinal |
Finding |
Points |
|---|---|---|
|
pseudosacculations |
absent |
0 |
|
|
present |
4 |
|
evidence of bowel dilatation on small bowel barium study and/or barium enema |
absent |
0 |
|
|
present |
2 |
|
fecal fat score on 100 gram fat diet |
<= 10 g/24 hr |
0 |
|
|
>10 to 20 g/24 hr |
2 |
|
|
> 20 g/24 hr |
3 |
maximum lower GI score = 9
|
Muscle |
Finding |
Points |
|---|---|---|
|
creatine kinase (CK) level |
|
as times the upper limit of normal |
|
myositis on EMG |
absent |
0 |
|
|
present |
2 |
|
myopathy on EMG |
absent |
0 |
|
|
present |
3 |
where:
• CK is referred to creatinine phosphokinase in the original table.
• CK level is entered as the percent of the upper limit of normal, then divided by 100 (for 100%). This is the number of multiples for the upper limit of normal.
maximum muscle score = none
|
Cardiac |
Finding |
Points |
|---|---|---|
|
cardiomegaly on chest X-ray, or moderate-to-large pericardial effusion on echocardiogram |
absent |
0 |
|
|
present |
1.5 |
|
symptomatic congestive heart failure |
absent |
0 |
|
|
present |
2 |
|
symptoms of arrhythmias |
absent |
0 |
|
|
present |
1.5 |
|
echocardiographic score |
|
0 - 2 |
where:
• Symptoms of arrhythmias: palpitations, syncope, fainting associated with ventricular tachycardia, or > 5 premature ventricular contractures (PVCs) on Holter monitor.
• To get a maximum of 7 points, the echocardiographic score must have a maximum of 2 points. The method of the score is not specified. I am implementing it as 1 point for right ventricular dilatation or hypertrophy and 1 point for left ventricular dilatation or hypertrophy.
maximum cardiac score = 7
|
Right Heart |
Finding |
Points |
|---|---|---|
|
accentuated second pulmonic sound on physical examination |
absent |
0 |
|
|
present |
2 |
|
right ventricular hypertrophy, right axis deviation, or right atrial enlargement on EKG |
absent |
0 |
|
|
present |
2 |
|
right bundle branch block on EKG |
absent |
0 |
|
|
present |
2 |
maximum right heart score = 6
|
Left Heart |
Finding |
Points |
|---|---|---|
|
left ventricular hypertrophy, left axis deviation, or left atrial enlargement on EKG |
absent |
0 |
|
|
present |
2 |
|
left bundle branch block on EKG |
absent |
0 |
|
|
present |
2 |
|
intraventricular conduction delay on EKG |
absent |
0 |
|
|
present |
2 |
|
myocardial infarction by history or on EKG |
absent |
0 |
|
|
present |
2 |
maximum left heart score = 8
|
Renal |
Finding |
Points |
|---|---|---|
|
renal crisis |
absent |
0 |
|
|
present |
10 |
|
proteinuria in mg/24 hours |
|
(value) / 150 |
|
creatinine clearance in mL/min |
|
100 / (value) |
where:
• For a creatinine clearance < 1, I set the points to 100.
maximum renal score = none
Purpose: To evaluate organ involvement in systemic sclerosis using the organ indices of Furst et al.
Specialty: Immunology/Rheumatology
Objective: laboratory tests, severity, prognosis, stage, response to therapy, disease progression
ICD-10: M34,
