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Assessment of Skin, Joint, Tendon and Muscle Involvement

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Assessment of Skin, Joint, Tendon and Muscle Involvement Assessment of skin, joint, tendon and muscle involvement A. Akesson1, G. Fiori2, T. Krieg3, F.H.J. van den Hoogen4, J.R. Seibold5 1Lund University Hospital, Lund, Sweden; ABSTRACT The extent of skin involvement is also 2Istituto di Clinica Medica IV, Florence, This rep o rt makes re c o m m e n d at i o n s the prime clinical criterion for the sub- Italy; 3University of Cologne, Koln, for standardized techniques of data ga - classification of SSc into its two princi- 4 Germany; University Medical Centre t h e ring and collection rega rd i n g : 1 ) pal subsets – SSc with diffuse cuta- St. Raboud, Nijmegen, The Netherlands; 5UMDNJ Scleroderma Program, skin involvement 2) joint and tendon in - neous involvement (diffuse scleroder- New Brunswick, New Jersey, USA. volvement, and 3) involvement of the ma) and SSc with limited cutaneous Anita Akesson, MD, PhD; Ginevra Fiori, skeletal muscles. The recommendations i nvo l vement (limited scl e ro d e rma – MD; Thomas Krieg, MD; Frank H.J. van in this report derive from a critical re - p rev i o u s ly termed the “CREST syn- den Hoogen, MD, PhD; James R. Seibold, v i ew of the ava i l able literat u re and drome”) (3). By consensus and conven- MD. group discussion. Committee re c o m - t i o n , p atients with skin invo l ve m e n t Please address correspondence to: mendations are considered appropriate restricted to sites distal to the elbows James R. Seibold, MD, Professor and for descri p t ive clinical inve s t i gat i o n , and knees, exclusive of the face, are Director, UMDNJ Scleroderma Program, translational studies and as standards considered to have limited scleroderma MEB 556 51 French Street, New for clinical practice. whereas patients with involvement of Brunswick, New Jersey 08903-0019, USA. Skin invo l vement should be assessed sites proximal to the elbows and knees E-mail: [email protected] using the modified Rodnan skin score. and inclusive of the chest and abdomen Clin Exp Rheumatol 2003; 21 (Suppl. 29): Joint involvement, when symmetric sy - are said to have diffuse scleroderma. S5-S8. n ovitis is pre s e n t , could be best as - Some investigato r s hold that an interme - © Copyright CLINICAL AND sessed by the DAS-28 as is utilized in di a te syndrom e ex i s t s , wh i ch is des- EXPERIMENTAL RHEUMATOLOGY 2003. r h e u m atoid art h ritis. Clinical assess - c ribed as skin invo l vement invo l v i n g Key words: Scleroderma, Rodnan ment should include a routinized evalu - the upper arms and thighs but sparing skin score, tender rubs, DAS-28. ation for the presence and number of the chest and abdomen (4, 5). palpable tendon friction rubs. Muscle This subclassification of SSc is closely involvement should be screened for by related to the time of onset, pace of performance of the serum creatine pho - development, and patterns of internal sphokinase assay and assessment of organ involvement and is accordingly proximal weakness. More specific test - strongly linked to survival. All descrip- ing including EMG, ma gnetic res o n a n c e tive clinical investigations in SSc today imaging and muscle biopsy should be should re q u i re the perfo rmance of a employed in those patients with clini - clinical assessment of the extent of skin cally significant myopathy only. involvement. Skin involvement Severity of change Tightening and thickening of the skin Severity of skin involvement is highly (scleroderma) is a cardinal clinical fea- relevant to the study of individuals with ture of systemic sclerosis (SSc). There diffuse scleroderma. Consensus obser- are rare patients with characteristic se- vations by veteran observers hold that rologic, vascular and visceral features skin ch a n ge in diffuse scl e ro d e rm a of SSc who lack skin thickening (sys- evolves in three sequential stages: 1) temic sclerosis sine scleroderma) and early progression, 2) plateau or stabi- t h e re are many other disord e rs only lization, and 3) late improvement (1, 3, superficially related to SSc in which 6 , 7). Early progre s s ive skin ch a n ge s skin thickening is a cardinal cl i n i c a l appear to correlate with both tissue and feature (1), as for example, scleredema systemic immune activation and in- adultorum or eosinophilic fasciitis. flammatory changes (1). The stabiliza- tion of skin involvement is associated Extent of change with evidence of reduced local and sys- The extent of skin involvement is the temic infl a m m ation. Later improve- single major criterion for the classifica- ment of skin involvement is thought to tion of SSc in comparison to other reflect an admixture of post-inflamma- major connective tissue disorders (2). tory and post-fibrotic atrophy as well as S-5 Skin, joint, tendon and muscle involvement in SSc / A. Akesson et al. the remodeling of previously fibrosed the local skin score should be recorded b reasts. The chest should be as- tissue. as 0. If the local skin is so tethered as to sessed with the subject in a sitting A variety of techniques have been inve- p re clude confident assessment, t h e position. stigated to estimate the degree of skin examiner should use his/her best judg- 6. Abdomen: Assess from the xiphoid thickening and/or tethering in discrete ment. to the pelvic brim. The ab d o m e n anatomic areas (6). The most widely Patients in whom areas of atrophic skin should be assessed with the subject used technique is the modified Rodnan (tethered, but not thickened) predomi- supine. skin score (mRSS) in which 17 body n ate are not considered ap p ro p ri at e 7. Thighs and Legs : Assess with the areas are examined by clinical palpa- choices for clinical trial participation su b ject lying down with the hips, tion and scored based on examiner jud- even if they might otherwise fulfill the knees and ankles comfort a bly fle xed . gement of skin thickness on a 4-point criteria for inclusion and not those for 8. Feet: Assess the dorsum only. Feet ordinal scale (0 = normal thickness; 1 = exclusion. should be examined with the subject mild thickening; 2 = moderate thicken- Experience has demonstrated that indi- lying down with the hips, k n e e s , ing; and 3 = severe thickening). The vidual investigators, while consistent, and ankles comfortably flexed. range of the mRSS is thus from 0 (no d i ffer in their rep o rting of the skin skin thickening) to 51 (grade 3 change score. Some are “maximizers” and as- Critique of the Modified Rodnan in all 17 body areas) (6-8). The areas sign scores to individual anat o m i c Skin Score examined in the mRSS scheme include areas according to the most severe local The mRSS is regarded as a measure of the right and left fingers, hands, fore- involvement. Others tend to “average” the degree of fibrosis, yet it does not a rm s , upper arm s , t h i g h s , l ower leg s skin involvement over a given surface discriminate between the contributions and feet, as well as the face, anterior area. For the purposes of multicenter to skin thickening of fibrosis, edema chest and abdomen. trials, investigators should be encour- and infl a m m ation. Ignoring the ele- The modified Rodnan skin score is rec- aged to score individual areas with a ment of tethering of the skin to deeper ommended as the core assessment score that is most representative at the structures can be seen as underestimat- technique for all international descrip- area under examination. For example, ing the total burden of skin involve- tive clinical investigations in SSc.The if the distal forearm has a patch consid- ment on the function and mobility of reasons for selection of the mRSS are ered 2+ whereas the remainder of the patients in the later atrophic stages of numerous and compelling. forearm is 1+, then 1+ would be the disease (9). most representative score. Alternative- There is controversy within the inves- Performance of the modified Rodnan ly, if the area rated 2+ was relatively tigative community as to the robustness Skin Score extensive, then 2+ would be the recom- of mRSS as a primary outcome mea- The mRSS can be easily taught and yet mended score for the forearm. sure. It is recognized that worsening its broad use in clinical settings outside Recommendations for patient position- skin scores are associated with a higher of the core of investigators who active- ing and assessment of the individual risk of renal invo l vement and deat h ly participate in trials has been limited. areas of examination are as follows: (10-12) and that improving the skin While inve s t i gator training sessions 1. Face: Assess the area between the score is associated with better function- have validated the utility of mRSS, the zygomatic arch and the lower man- al capacity and surv ival (13). Th e s e fo l l owing practical guidelines have dible. Do not assess the forehead. data argue that the mRSS is a surrogate evolved that enhance the understanding 2. Fingers: Concentrate on the skin of but perhaps not a primary measure of and application of the measure. We be- the dorsum of the fingers. Do not outcome.
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