Tools That Assess Sarcoidosis Severity Should See More Use Across Specialties

A study in JAMA Dermatology provides evidence that the use of two tests to assess cutaneous sarcoidosis disease severity should be expanded. The research has applications for dermatologists, rheumatologists, and pulmonologists, all of whom have a role in treating sarcoidosis.

Because sarcoidosis — an uncommon, multisystem inflammatory disease characterized by noncaseating granulomatous infiltrates – generally involves a great deal of care coordination, the researchers argue, a criterion standard outcome measure for the cutaneous manifestations of sarcoidosis should align and agree.

“In the absence of a standardized, validated, and widely accepted instrument to measure cutaneous sarcoidosis severity, many … clinical trials [have] used different ad hoc outcome instruments to assess objective clinical severity and therapeutic end points, thus limiting the validity of the results and comparability across trials,” the study authors wrote.

The cross-sectional study involved four dermatologists, three pulmonologists, and four rheumatologists with experience in diagnosing and managing sarcoidosis as well as 13 patients from the Cutaneous Sarcoidosis Clinic at the Hospital of the University of Pennsylvania. All eligible patients had a clinical diagnosis of cutaneous sarcoidosis with lesions on the face. The study analyzed the use of the Cutaneous Sarcoidosis Activity and Morphology Instrument (CSAMI) and Sarcoidosis Activity and Severity Index (SASI), using the Physician’s Global Assessment (PGA) as a reference instrument. Interrater reliability was good for the CSAMI Activity scale (0.69 [0.51-0.87]) and PGA (0.66 [0.47-0.85]), weak for the CSAMI Damage scale (0.26 [0.11-0.52]), and excellent for the modified Facial SASI (0.78 [0.63-0.91]).

“This study was unique in its inclusion of pulmonologists, rheumatologists, and dermatologists who treat patients with sarcoidosis… These results lend credibility to expand the use of the CSAMI and SASI by dermatologists and nondermatologists in assessing cutaneous sarcoidosis disease activity,” the authors wrote.

As expected, the CSAMI Damage scale did not correlate well with the PGA, but the study authors explained that this was because the CSAMI Damage scale is designed to capture residual skin damage from prior disease activity and not currently active disease. The PGA, however, is generally used to measure current overall disease activity.

One key aspect of the study is the idea that an ideal outcome instrument — particularly in dermatology – should give a picture of the disease characteristics not only from a clinician’s perspective but also one that reflects patients’ subjective impressions on the effect of disease on their lives.

“This combination is essential for the development of clinically relevant and patient-oriented end points for interventional trials and clinical practice,” the researchers observed. “Overall, our results provide additional support for the construct validity of the CSAMI in reflecting physician- and patient-assessed disease severity measures, which should be confirmed in future research.”

Study limitations include the small sample size and the fact that the use of the CSAMI and SASI for nonfacial lesions has not been validated specifically among non-dermatologists. The SASI tool, in particular, has recently been modified in clinical trials to evaluate nonfacial lesions; the study authors would like to see outcomes from this modified version.