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Abstract Number: 1811

Validation Of The ICD-CM-9 Code For Systemic Sclerosis Using Updated ACR/EULAR Classification Criteria

Aaliya Yaqub1, Lorinda Chung2, David Fiorentino3 and Eswar Krishnan4, 1Stanford Univ Medical Center, Stanford, CA, 2Immunology and Rheumatology, Stanford University School of Medicine, Palo Alto, CA, 3Dermatology, Stanford University School of Medicine, Redwood City, CA, 4Medicine, Stanford University, Palo Alto, CA

Meeting: 2013 ACR/ARHP Annual Meeting

Keywords: ICD-9, scleroderma and systemic sclerosis

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Session Information

Title: Systemic Sclerosis, Fibrosing Syndromes, and Raynaud’s - Clinical Aspects and Therapeutics II

Session Type: Abstract Submissions (ACR)

Background/Purpose: Systemic sclerosis (SSc) is a rare autoimmune disease associated with substantial morbidity.  Epidemiologic studies using large administrative databases often rely on the accuracy of International Classification of Diseases-Clinical Modification-9 (ICD-CM-9) codes for case definitions of medical conditions such as SSc. A previous study of 89 hospitalized patients at a tertiary care center found that the accuracy of ICD-CM-9 code 710.1 for determining true cases of SSc that met the 1980 ACR criteria was 45%.  We sought to determine the accuracy of the ICD-CM-9 code of 710.1 for identifying cases of SSc using a large outpatient cohort of well-characterized patients.

Methods: This was a retrospective analysis of the medical records of all patients evaluated at Stanford Hospital and Clinics from 2005-2012 who have an associated ICD-CM-9 code of 710.1.  True cases of SSc were defined as any of the following: a) Fulfill the 1980 American College of Rheumatology (ACR) classification criteria for SSc; b) Have at least 3 of 5 of the CREST (calcinosis, Raynaud’s, esophageal dysmotility, sclerodactyly, telangiectasias) features; OR c) Fulfill preliminary ACR/European League Against Rheumatism (EULAR) revised criteria for the classification of SSc.

Results: 432 patient records were reviewed.  Mean age was 57±14.5 years, 84% were female, 51% Caucasian.  21% had diffuse cutaneous disease, 66% had limited disease, and 3% were characterized as SSc sine sclerosis.  238 (55%) patients met the 1980 ACR criteria, 292 (68%) patients met CREST criteria, and 297 (69%) patients met the revised ACR/EULAR criteria. The accuracy of ICD-9-CM code 710.1 for determining true cases of SSc by any of the three classification criteria was 77%.  The ICD-CM-9 code for SSc was accurate 71% of the time when the 1980 ACR criteria were applied, and 75% of the time when the CREST or ACR/EULAR revised criteria were applied.

Table 1: Clinical Characteristics of 432 Systemic Sclerosis Patients by Criteria


Classification Criteria

Percentage of patients affected

 

 

1980 ACR Criteria:

 

Major criterion:

 

Proximal diffuse (truncal) sclerosis (skin tightness, thickening, non-pitting induration)

42%

 

 

Minor criteria: 

 

  • Sclerodactyly (only fingers and/or toes)

63%

  • Digital pitting scars or loss of substance of the digital finger pads (pulp loss)

25%

  • Bilateral basilar pulmonary fibrosis

29%

 

 

Patients who met the major criterion or 2 minor criteria:

55%

 

 

CREST Criteria

 

 

 

Calcinosis

12%

Raynaud’s Phenomenon

82%

Esophageal dysmotility

60%

Sclerodactyly

63%

Telangiectasias

49%

 

 

Patients who met 3 of 5 CREST criteria:   

68%

 

 

Revised ACR/EULAR Criteria

 

 

 

Skin thickening of the fingers

 

puffy fingers

25%

whole finger distal to MCP

47%

 

 

Fingertip lesions

 

digital ulcers

28%

pitting scars

21%

 

 

Abnormal nailfold capillaries

33%

 

 

Pulmonary involvement

 

Pulmonary hypertension

20%

Interstitial lung disease

29%

 

 

Autoantibody profile

 

Positive ANA

62%

Positive scl-70 Ab

11%

Positive anti-centromere Ab

20%

 

 

Patients who scored ≥ 9 points in the revised ACR/EULAR criteria:

69%

 

 

*Raynaud’s, telangiectasia percentages already noted above.

 

Conclusion:

We found an acceptable accuracy rate of 77% for the ICD-CM-9 code 710.1 for determining true cases of SSc according to any of 3 different classification schemes. Our study supports the validity of this ICD-CM-9 code for identifying cases of SSc for epidemiologic studies using administrative databases.


Disclosure:

A. Yaqub,
None;

L. Chung,
None;

D. Fiorentino,
None;

E. Krishnan,

Takeda,

2,

takeda,

5.

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