Clinical study Esophageal motility in scleroderma Esophageal motllity abnormalities in sckroderma. Report of 17 patients C. Massone, P.L. Bozzano, A. Paradi, N. Pandolfo andA. Rebora ABSTRACT Objective: Aim of this work was to study esophageal motor abnormalities in patients with systemic sclerosis (SSc). Patients and methods: We studied 17 consecutive patients with SSc classified according to Le Roy et al. In each patient we performed barium esophagograms and esophageal manometry (EM) and we evaluated in particular lower esophageal sphincter (LES) pressure and relaxation and esophageal peristalsis. We also calculated the differences between the esophageal and intragastric pressure. Results: Esophageal abnormalities were detected by EM in all patients. Wave amplitude and duration were decreased in 14 and 9 patients respectively. Wave propagation was abnormal in 11 patients and LES pressure was reduced in all but 1. Only two patients with edematous skin changes and 1-year-long Raynaud's phenomenon had a slight esophageal involvement. Conclusion: Esophageal involvement was detected in all patients even though asymptomatic. A relationship was noted with the duration of the disease, but not with the severity of skin involvement. Introduction Esophageal motility abnormalitles occur in up to 90% ofthe patients with systemic sclerosis (SSc), about half of them being asymptomatic. The relationship of such disturbances with the duration of the disease and w ith SSc specific serology is poorly understood. In this study we compared the esophageal abnormalities with the extent of skin involvement and the disease duration in 17 consecutive SSc patients. Patients and methods We studied 14 females and 3 males, mean age 56. According to Le Roy et al (1) they were classified as having limited SSc (ISSc, 14 patients) ·and diffuse SSc (dSSc, 3 patients). Each patient had a complete history and physical examination. In particular, dysphagia, heartburn, vomiting and substernal pain were recorded. Antinuclear antibodies (ANA) and anti-centromere antibodies (ACA) were detected by indirect immuno- acta dermatovenerologica A.P.A. Vol 8, 99, No 4 --------------- --- ---------- - 141 Esophageal mmility in scleroderma Table 1. Criteria for scoring symptoms in patients with limited and diffuse systemic sclerosis. Scores s3 slight, 4-7 mild, >7 severe involvement. Medium Normal wave amplitude(> 25 mmHg) o Mildly reduced wave amplitude C 10-25 mmHg) 1 Severely reduced wave amplitude C< 10 mmHg) or aperistalsis 2 Lower Normal wave amplitude (> 25 mmHg) o Mildly reduced wave amplitude C 10-25 mmHg) 1 Severely reduced wave amplitude ( < 10 mmHg) or aperistalsis 2 Medium Normal wave duration (3-6 seconds) o Abnormal wave duration C< 3 or > 6 seconds) 1 Lower Normal wave duration (3-6 seconds) o Abnormal wave duration ( < 3 or > 6 seconds) 1 Propagated ~ 75% o wave 50-75% 1 s50% 2 Lower t-.P normal (18-22mmHg) o esophageal t-.P mildly reduced (11-17 mmHg) 1 sphincter t-.P severely reduced (s lOmmhg) 2 Tota! possible score: 10 fluorescence (IIF) , anti Sel-70 antibodies were detected by immunodiffusion assay and ELISA. All patients underwent barium esophagograms ancl esophageal manometry (EM). The latter was performed by pneumohydraulic capillary infusion system (Arndofer type) (2). We evaluated the pressure of the upper and lower esophageal sphincters (UES-LES) and their relaxation. Esophageal peristalsis was studied by calcu- lating the wave amplitude, duration and propagation. Moreover, we calculated the differences between the esophageal and intragastric pressure (t-. P) and we measmed the abdominal length ofLES. As SSc affects the distal two-thirds of the esophagus, we considered only abnormalities of these segments. To grade the esophageal motility abnormalities, we appliecl an arbitrary 7-point score scale (Table 1). A score s 3 clocumented a slight esophageal involvement, 4-7 a mild involvement and >7 a severe one. Results Fourteen patients hacl lSSc ancl 3 dSSc. The skin exhibitecl edematous changes in 6 patients , selero- eclematous in 6 ancl selerotic in 5. Teleangiectases, qlcinosis and ulcerations were present in 15, 11 and 6 patients respectively. Pulmonar, renal and cardiac invol- vements were present in 12, 7 and 6 patients respec- tively. One patient hacl primary bilia1y cirrhosis, 1 Parkin- son's clisease and 2 Sjogren's synclrome. Only 2 patients hacl anti Sel-70 antibodies, 10 had ACA ancl the remainders speckled pattern of ANA. Heartburn was present in 11 patients , dysphagia in 7 and regurgitation in 7. Only 4 patients were completely asymptomatic. Esophageal abnormalities of some type were detec- teci by EM in all patient~. In particular, UES pressure and coordination with pharynx were normal in all patients but 1, ancl wave amplitude ancl cluration were decreased in the 7 1 year 1 year 10 years 10 years 16 years 10 years 10 years 7 years 30 years 28 years 5 years 2 years 10 years 10 years 20 years 55 years 40 years Years indicate Raynaud's phenomenon duration * this patient was Sel-70 positive and had dSSc Discussion EM proved to be the most sensitive method to detect esophageal hypomotility (3,4) being positive even in asymptomatic patients. Our study confirms that eso- phageal involvement is very common in SSc. As pre- viously described, incoordination, reduction and abnor- mal duration of the waves and failure of the LES to relax are its main features . Our study also confirms that there is no relationship between the degree of esophageal dysfunction and the severity of skin changes (5) or the presence of a particular ANA. to depend on the duration of the disease. The 2 patients with a slight involvement of esophagus (::;3 score) had only edematous skin and experienced Raynaud's pheno- menon only since 1 year. The other 4 patients with ede- matous skin changes and esophageal score >4 had Ray- naud's phenomenon for 10 years or more. Moreover, all but one of the patients with scleroedematous and sclerotic skin changes had an esophageal score >4 and Raynaud's phenomenon for 21 years, as an average. In conclusion, esophageal involvement was detected in all patients even though asymptomatic. Motor abnor- malities seem to parallel the duration of the disease , but not the severity of the skin involvement. In our study, the esophagus involvement seemms REFEHENCES AUTHORS' ADDRESSES 1) Le Roy EC, Block C, Fleishmajer Ret al: Scleroderma (Systemic Sclerosis) : classification subsets and pathogenesis. J Rheumatol 1988; 15: 202-5. 2) Spigno L, Pandolfo N, Guiddo G et al. Analisi computerizzata della manometria esofagea. Min Chir 1991; 46: 27-35. 3) Kaye SA, Siraj QH, Agnew J et al. Detection of early asymptomatic esophageal dysfunction in systemic sclerosis using a new scintigraphic grading method. J Rheumatol 1996; 23: 297-301. 4) Lapadula G, Muolo P, Semeraro F et al. Esophageal motility disorders in the rheumatic diseases: a review of 150 patients. Ciin Exp Rheumatol 1994; 12: 515-21. 5) Sjogren RW: Gastrointestinal features of scleroderma. Curr Op Rheumatol 1996; 8: 569-75. Cesare Massone, MD, DISEM, Section oj Dermatology, University oj Genoa, V. Benedetto XV n. 7 1613 2, Genoa, Jtaly Alessandra Paradi, MD, same address .. A. Rebora, MD, same address Pier Luigi Bozzano, MD, DISEM, Department oj Surgery, University oj Genoa, V. BenedettoXVn. 7 16132, Genoa, Italy Nico la Pandolfo, MD, same address . acta dermatovenerologica A.P.A. Vol 8, 99, No 4 - ------------------------ - ---- 143