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CASE REPORT

Dysphagia as an Early Presenting Symptom in Dermatomyositis

Adeputri Tanesha Idhayu*, Marcellus Simadibrata**, Gerie Amarendra* *Department of Internal Medicine, Faculty of Medicine, University of Indonesia Dr. Cipto Mangunkusumo General National Hospital, Jakarta **Division of , Department of Internal Medicine, Faculty of Medicine University of Indonesia/Dr. Cipto Mangunkusumo General National Hospital, Jakarta

ABSTRACT Dermatomyositis is a systemic disorder that frequently affects the esophagus, lungs, and the heart. Dermatomyositis diagnostic criteria involve evaluation of proximal muscle weakness, elevation serum levels of muscle enzyme, characteristic features of , typical muscle biopsy and classical skin rash of dermatomyositis. The prevalence of in patients with dermatomyositis varied from 10-73%. $\HDUROGPDOHZDVDGPLWWHGWR&LSWR0DQJXQNXVXPRKRVSLWDOZLWKFKLHIFRPSODLQWRIGLI¿FXOWLQ . He had been well until he began to have muscle weakness, myalgia, fatigue, and obstructive symptom in his upper digestion tract. Physical examination showed general weakness and symmetrical rash on the face, chest and back. Laboratory examination revealed anemia, thrombocytopenia, and elevated transaminase serum level. Antinuclear antibody was positive. Esophagogastroduodenoscopy showed severe esophagitis. The gastric mucosa biopsy revealed non-active chronic gastritis, antral lymph atrophy, and non-dysplastic. Biopsy of esophageal mucosa showed Barret’s esophagus with squamous epithelial cell and hard dysplasia focus. The electromyography result was suspected to a dermatomyositis. The deltoid muscle biopsy demonstrated dystrophy. Dysphagia may be an initial presenting symptom and especially prevalent in patients with dermatomyositis RURWKHULQÀDPPDWRU\P\RSDWK\'\VSKDJLDDVVRFLDWHGZLWKWKHVHP\RSDWKLHVSULPDULO\DIIHFWVWKHVNHOHWDO muscle–activated oropharyngeal phase of swallowing. It may precede weakness of the extremities or present DVWKHVROHV\PSWRP5HFRPPHQGHGWUHDWPHQWVXVHGWRWUHDWLQÀDPPDWRU\P\RSDWK\DVVRFLDWHGG\VSKDJLD DUHFRPELQDWLRQRIPHGLFDOUHKDELOLWDWLRQDQGLQWHUYHQWLRQDO'\VSKDJLDDVVRFLDWHGZLWKQXWULWLRQDOGH¿FLWV DVSLUDWLRQSQHXPRQLDGHFUHDVHGTXDOLW\RIOLIHDQGSRRUSURJQRVLV3DWLHQWVZLWKLQÀDPPDWRU\P\RSDWK\DQG dysphagia are reported to have a 1-year mortality rate of 31%.

Keywords:G\VSKDJLDGHUPDWRP\RVLWLVLQÀDPPDWRU\P\RSDWK\

ABSTRAK Dermatomiositis merupakan penyakit sistemik yang sering menyerang esofagus, paru-paru, dan jantung. Kriteria diagnosis dermatomiositis melibatkan kelemahan otot proksimal, elevasi serum enzim otot, gambaran HOHNWURPLRJUD¿\DQJNKDVELRSVLRWRW\DQJWLSLNDOGDQUXDPNXOLW\DQJNKDVGHUPDWRPLRVLWLV3UHYDOHQVLGLVIDJLD pada pasien dengan dermatomiositis bervariasi antara 10-73%. Pada kasus ini dilaporkan seorang pria berusia 40 tahun dirawat di rumah sakit Cipto Mangunkusumo dengan keluhan utama sulit menelan. Keluhan diawali dari rasa lemah tubuh, mialgia, kelelahan, dan gejala obstruktif GLVDOXUDQSHQFHUQDDQDWDV3HPHULNVDDQ¿VLNPHQXQMXNNDQNHOHPDKDQVHOXUXKWXEXKGDQUXDPVLPHWULVSDGD wajah, dada dan punggung. Pemeriksaan laboratorium menunjukkan anemia, trombositopenia, peningkatan enzim transaminase, dan antibodi antinuklear positif. Endoskopi saluran cerna bagian atas menunjukkan HVRIDJLWLVEHUDW%LRSVLPXNRVDODPEXQJPHQXQMXNNDQJDVWULWLVNURQLV\DQJQRQDNWLIDWUR¿NHOHQMDUJHWDK bening di antrum, dan non displasia pada mukosa. Biopsi mukosa esofagus menunjukkan Barret esophagus GHQJDQVHOHSLWHOVNXDPRVDGDQIRNXVGLVSODVLDNHUDV+DVLOHOHNWURPLRJUD¿PHQXQMXNNDQNHFXULJDDQNHDUDK GHUPDWRPLRVLWLVGDQKDVLOELRSVLRWRWGHOWRLGPHQXQMXNNDQGLVWUR¿ Disfagia dapat menjadi gejala awal atau merupakan keluhan utama dari dermatomiositis atau miopati LQÀDPDVLODLQQ\D'LVIDJLDEHUKXEXQJDQGHQJDQPLRSDWL\DQJPHPSHQJDUXKLRWRWUDQJND\DQJPHQJDNWLINDQ

56 The Indonesian Journal of Gastroenterology, Hepatology, and Digestive Endoscopy Volume 12, Number 1, April 2011 57 Adeputri Tanesha Idhayu, Marcellus Simadibrata, Gerie Amarendra fase orofaringeal pada proses menelan. Keluhan disfagia dapat mendahului gejala kelemahan ekstremitas atau sebagai gejala tunggal pada dermatomiositis. Rekomendasi terapi untuk tatalaksana disfagia pada miopati LQÀDPDVLPHUXSDNDQNRPELQDVLGDULREDWREDWDQUHKDELOLWDVLGDQWHUDSLLQWHUYHQVL'LVIDJLDEHUKXEXQJDQ GHQJDQWHUMDGLQ\DGH¿VLWQXWULVLSQHXPRQLDDVSLUDVLPHQXUXQQ\DNXDOLWDVKLGXSGDQSURJQRVLV\DQJEXUXN 3DVLHQGHQJDQLQÀDPDVLPLRSDWLGDQGLVIDJLDGLODSRUNDQPHPLOLNLPRUWDOLWDVWDKXQVHEHVDU

Kata kunciGLVIDJLDGHUPDWRPLRVLWLVPLRSDWLLQÀDPDVL

INTRODUCTION GLI¿FXOW\VZDOORZLQJ+HKDGEHHQZHOOXQWLORQH Inflammatory myopathies are acquired muscle month later, and then he began to have muscle diseases that are typically categorized into 4 groups; weakness. He felt myalgia and fatigue, as well as loss including polymyositis (PM), dermatomyositis (DM), of appetite; and he had lost 5 kg of weight. Two weeks inclusion body myositis (IBM), and an overlap syndrome before admission, he felt an obstruction developed in with mixed characteristics. DM is an idiopathic KLVXSSHUGLJHVWLRQWUDFWZKLFKPDGHKLPKDGGLI¿FXOW\ LQÀDPPDWRU\P\RSDWK\ZLWKFRPPRQFOLQLFDOIHDWXUHV swallowing. It was accompanied with a dry cough, of slowly progressive symmetrical muscle weakness and hoarseness and . There was no chest cutaneus manifestations. The pathogenesis of cutaneus SDLQQRGLI¿FXOW\WREUHDWKQRGLI¿FXOW\WRSDVVXULQH disease of DM is poorly understood; however the and no or . following factors have been implicated. DM rarely occurs Since 6 months prior to admission, the patient found in multiply members but may be linked to certain human a pruritic-papular rash in the face and then new rashes leukocyte antigen (HLA), immunological abnormalities, were developed along upper portion of the chest and viral infection, exposure to ultraviolet light, drug-induced back and the color became brighten. There was no myopathies and malignancy.1 history of hair loss, no oral ulcer, no chest , and DM can occur in people of any age. DM affects both QRGLI¿FXOW\LQEUHDWKLQJ children and adults, and female are more often than The symptoms persisted and worsen, patient could PDOH'LDJQRVWLFFULWHULDDQGDFODVVL¿FDWLRQVFKHPH only eat soft and watery food, sometimes also get were based upon a combination of clinical, laboratory, hiccup. Subsequently, the patient went to Division of pathological evaluation. DM diagnostic criteria Gastroenterology, Department of Internal Medicine involves evaluation of proximal muscle weakness, at Cipto Mangunkusumo hospital, and then was elevation serum levels of enzyme, characteristic planned to have esophagogastro-duodenoscopy (EGD). features of electromyography (EMG), typical muscle +RZHYHUZKHQWKHIDWLJXHDQGGLI¿FXOW\VZDOORZLQJ biopsy histopathology, and the classical skin rash of were getting worse, he came to the emergency unit. DM. Dysphagia has been reported to occur in 10-73% At the emergency unit, the patient consumed food of these patients.1,2,3 through nasogastric tube. The patient was consulted to the The dysphagia associated with these myopathies Department of Ear, Nose and Throat and Department of primarily affects the skeletal muscle–activated Dermatology. Both departments concluded neurogenic oropharyngeal phase of swallowing. It may precede dysphagia and seborrheic dermatitis. weakness of the extremities or present as the sole There was no history of heart problem, pulmonary symptom. Dysphagia is associated with nutritional problem, and no history of allergy of food or medicine. deficits, aspiration , decreased quality He denied the same problem in her family. Patient was of life, and poor prognosis. In fact, patients with a public transportation driver and had been exposed to LQÀDPPDWRU\P\RSDWK\DQGG\VSKDJLDDUHUHSRUWHG concrete dust and radiation. He had a 20-year smoking to have a 1-year mortality rate of 31%. A variety of history. He had no recent exposure to ill persons and medical, rehabilitation, and interventional treatments had not traveled recently. DUHXVHGWRWUHDWLQÀDPPDWRU\P\RSDWK\DVVRFLDWHG Based on physical examination, the patient was dysphagia. The following case report presents a case of appeared ill, compos mentis, and malnourished. 40-years-old man with dysphagia as an early presenting The blood pressure was 100/60 mmHg, pulse rate symptom, which did not occur long enough for us to ZDVEHDWVPLQXWHERG\WHPSHUDWXUHZDVÛ& recognize that the patient’s diagnosis was DM.1,4 body weight was 43 kg, height was 160 cm and body mass index was 16.79 kg/m2. On skin examination, CASE ILLUSTRATION we revealed rashes, which were symmetrical, A male, 40 years old, was admitted to Cipto erythematous, lenticular, placate with squama on the Mangunkusumo hospital with chief complaint of facial (ala nasi, naso-labial and bucal region), scalp, and neck. The conjunctiva was not pale, no .

58 The Indonesian Journal of Gastroenterology, Hepatology, and Digestive Endoscopy Dysphagia as an Early Presenting Symptom in Dermatomyositis

Her lung sounded vesicular, which were normal. were normal. Electrocardiography (ECG) result was Neither rales nor wheezing was found. Her 1st and 2nd ZLWKLQQRUPDOOLPLWFKHVW;UD\VKRZHGQRLQ¿OWUDWH heart sound were normal, no murmur or gallop was no cardiomegaly. The hepatic seromarker was positive found. Our abdominal examination found no distention for hepatitis B surface antigen (HBsAg) and negative and no tenderness. The and spleen were not for anti hepatitis C virus (HCV). palpable. No edema was found on both extremities. The gastric mucosa biopsy demonstrated non Laboratory examination revealed hemoglobin level active chronic gastritis, antral lymph atrophy, of 10.9 g/dL, hematocryt of 34.6%, the leukocyte non-dysplastic and found no Helicobacter pylori. count was 5,010/uL, platelet count was 89,000/uL, The liver biopsy demonstrated chronic hepatitis, erythrocyte sedimentation rate (ESR) was 50 mm/ piecemeal mild necrosis, focal moderate necrosis and KRXUPHDQFRUSXVFXODUYROXPH 0&9 ZDVÀ ¿EURVLVVWDJLQJ)(YDOXDWLRQRI(*'GHPRQVWUDWHG mean corpuscular hemoglobin (MCH) was 27 pg, grade C esophagitis with mild gastritis. mean corpuscular hemoglobin concentration (MCHC) The esophagus mucosa biopsy demonstrated was 34 g/dL. The differential count showed following Barret’s esophagus with squamous epithelial cells results: eosinophil 0, basophil 0, neutrophil 51.9%, with hard dysplasia focus. The EMG demonstrated lymphocytes 10%, and monocytes 3%. The peripheral myogenic diffuse lesion with motor neuropathy EORRG¿OPVKRZHGQRUPRF\WLFQRUPRFKURPLFDQHPLD axonal type in right ulnar nerve and left sural nerve, ureum level was 17 mg/dL, creatinine level was which were suspected to a DM. The patient underwent 0.3 mg/dL, aspartate aminotransferase (AST) level a deltoid muscle biopsy which demonstrated dystrophy. was 100 U/L, alanine aminotransferase (ALT) level Antinuclear antibody (ANA) was positive and all was 40 U/L, albumin level was 2.70, globulin level SUR¿OHRI$1$LQFOXGLQJDQWL-RZHUHQHJDWLYH was 3.00, creatine kinase (CK) level was 300 U/L, 7KH&.ZDV8/7KHKLVWRU\FOLQLFDO¿QGLQJV blood glucose level was 92 mg/dL, sodium level and blood tests all supported to a DM and therefore was 135 mEq/L, kalium level was 3.9 mEq/L, and methylprednisolone tablet 3 times 16 mg/day was chloride level was 97.1 mEq/L. Urine analysis results commenced.

Figure 1. The barium-swallow X-rays with contrast showed normal oesophageal motility

Figure 2. Endogastroduodenoscopy showed severe esophagitis with multiple ulcers in corpus and antrum

Volume 12, Number 1, April 2011 59 Adeputri Tanesha Idhayu, Marcellus Simadibrata, Gerie Amarendra

The patient was discharged 60 days later with Such criteria should be applied assuming that VLJQL¿FDQWLPSURYHPHQWLQV\PSWRPVDQGSODQQHG the possibilities of infectious, toxic, metabolic, to be followed up by both the gastroenterologists and dystrophic or endocrine myopathies have been excluded rheumatologists in daily clinic. by appropriate evaluation. Several sets of criteria have EHHQSURSRVHGIRUWKHGLDJQRVLVDQGFODVVL¿FDWLRQRI DISCUSSION LGLRSDWKLFLQÀDPPDWRU\P\RSDWKLHV$OORIWKHPKDYH The idiopathic inflammatory myopathies are limitation and none have been properly validated. rare diseases. The prevalence of the inflammatory Despite their limitation, the Bohan and Peter criteria myopathies is estimated at 1 in 100,000. PM as a stand- RIWHQXVHGIRUGLDJQRVLVDQGFODVVL¿FDWLRQRILGLRSDWKLF alone entity is a rare disease affecting adults. DM affects LQÀDPPDWRU\P\RSDWKLHV12 both children and adults, and women are more often than Based on the history of this patient, we found men. IBM is three times more frequent in men than in GLI¿FXOWVZDOORZLQJZLWKV\PPHWULFDODQGELODWHUDO women, more common in Caucasians than blacks, and muscle weakness since 1 month prior to admission. is most likely to affect persons > 50 years of age. He felt myalgia and muscle weakness that had worsen The mechanisms that cause autoimmune day by day. No history of oral ulcer, pain and joint reaction have not been known, but both genetic and swelling. Six months prior to admission, he felt altered environmental factors are likely to confer risk factors VNLQFRQGLWLRQDFFRPSDQLHGZLWKLWFKLQJ)LUVWWKH GHYHORSLQJFKURQLFLQÀDPPDWRU\P\RSDWKLHV5HFHQW rash was on his face, then it ascended to upper portion studies on pathogenesis of the myopathy have been of the chest and back and the color became brighter. controversial. Some suggest that the myopathy in DM It was also accompanied with a dry cough, hoarseness is probably caused by complement-mediated (terminal and hypersalivation. We suggested such condition as DWWDFNFRPSOH[ YDVFXODULQÀDPPDWLRQ7KHVWURQJHVW the signs of dermatomyosities. genetic association is HLA; while the most frequently Based on physical examination, we found associated environmental factor is viral infection. pathognomonic or typical rash for dermatomyositis, Other observations suggest the correlation between which was similar to the heliotrope rash and ultraviolet light exposure and myositis. Several drug poikiloderma that occurred in a V-shaped distribution may induce myopathies and increase the risk of having over the anterior neck, upper chest and back region malignancy for patient with DM.2,5,6,7,8 (the shawl sign). In some cases, it may dominate Inflammatory myopathies are acquired muscle the clinical symptoms. The skin rash may precede diseases that are typically categorized into 4 groups: the muscle symptoms by months or even years. In some PM, DM, IBM, and an overlap syndrome with mixed other patients, the skin manifestations may be the only characteristics. The clinically suspected diagnosis clinical sign of DM. Another characteristic skin rash in RI30'0RU,%0LVFRQ¿UPHGE\H[DPLQLQJWKH DM are the Gottron papules, that were found over bony VHUXPOHYHOVRIPXVFOHHQ]\PHV(0*¿QGLQJVDQG prominences, particularly the metacarpophalangeal muscle biopsy.3,9 joints, the proximal interphalangeal joints and/or DM is an idiopthic inflammatory myopathy, the distal interphalangeal joints, peri-ungual erythema, systemic disorders with characteristics of bilateral and calcinosis; which were not found in this patient. symmetrical muscle weakness and typical cutaneus Others clinical features for DM include arthralgia and ¿QGLQJV10,11,127KHLQÀDPPDWRU\P\RSDWKLHVZKLFK DUWKULWLVOXQJ¿EURVLVFDUGLRP\RSDWK\5D\QRXQG¶V are collectively often named as myositis, have common phenomenon and Sjogren syndrome. Dermatomyositis clinical features of slowly progressive symmetrical has been linked to internal malignancy in 15-25% muscle weakness, decreased muscular endurance and cases.3,4,12,13,14 fatigue.6 Based on the laboratory results, we found The diagnostic of myositis should be suspected in normocytic anemia, increased ESR up to 50 mm/hour, adult with sub-acute or insidious onset of symmetrical increased AST level of 100 U/L, increased ALT level of proximal muscle weakness. A diagnosis of myositis is 40 U/L, and increased CK level of 300 U/L. ANA was supported by typical laboratory results of myopathy such SRVLWLYHEXWDOOSUR¿OHRI$1$LQFOXGLQJDQWL-R as raised serum levels of CK or lactate dehydrogenase were negative. Autoantibodies are frequently found (LDH), myopathic change on EMG or a muscle in patients with myositis. Antinuclear antibodies were ELRSV\ZLWKVLJQRIP\RSDWK\$GH¿QLWHGLDJQRVLV found positive in 30% DM patients and 90-100% in can be made when histopathological changes present patients with systemic lupus erythematosus (SLE). LQPXVFOHELRSV\7KHVHFKDQJHVLQFOXGHLQ¿OWUDWHV A number of autoantibodies in DM have been detected, RIPRQRQXFOHDULQÀDPPDWRU\FHOOVUHJHQHUDWLQJDQG LQFOXGLQJ-RDQGDQWL0DVP\RVLWLVDQWLERGLHV GHJHQHUDWLQJPXVFOH¿EUHV2,6,12 ZHUHGLUHFWHGDVF\WRSODVPLFDQWLJHQ$QWL-RZDV

60 The Indonesian Journal of Gastroenterology, Hepatology, and Digestive Endoscopy Dysphagia as an Early Presenting Symptom in Dermatomyositis

SRVLWLYHLQRISDWLHQWVZLWK'0$QWL-RLV The skeletal-muscle activated oropharyngeal associated with myositis and pulmonary involvement phase of swallowing is clearly affected, which lead and is not the only marker of disease.7,14,15 We also to the increased incidence of . found hypoalbuminemia (2.70) with albumin globulin It finally results in ineffective peristalsis and ratio less than 1. Positive HBsAg was also found that FOLQLFDOPDQLIHVWDWLRQRIJDVWURRHVRSKDJHDOUHÀX[ we assessed as chronic hepatitis B. gastroparesis and constipation due to colonic inertia. The most important investigation were the A multi-disciplinary approach is essential and measurement for serum levels of muscle enzymes, the roles of dieticians, speech and language therapists EMG, and muscle biopsy. CK was considered as DUHLPSHUDWLYH$VVHVVPHQWZLWKYLGHRÀXRURVFRSLF the most sensitive and useful serum muscle enzyme studies can help us to identify pharyngeal pooling, WRPHDVXUHDQGPD\EHXVHGLQ¿QGLQJDQGDVVHVLQJ impaired -base retraction, decreased laryngeal WKHSURJUHVVRIWKHGLVHDVH&.LVQRWVSHFL¿FIRUP\RVLWLV elevation and cricopharyngeal dysfunction. Esophageal and may be seen in several other conditions. About and gastric radionuclide transit studies are safe, simple, 10-20% patients may have CK level within normal non-invasive method of highlighting esophageal and range. Other commonly used enzyme that may have gastric delay.1,4,5 elevated level in the serum for myopathic condition Principal treatment for DM is immunosuppressive 3,12,13,15 are ESR, LDH, AST, ALT. drugs. Treatment with glucocorticoid provides Patients with DM may mimic SLE in demostrating a substantial improvement in survival and reducing photosensitivity and malar rash. Skin biopsy in these disability. High initial dose of glucocorticoid are two diseases share similar histophatological feature recommended. Large prospective randomized of “interface dermatomyositis”. Skin biopsy in such controlled studies of steroids in polymyositis and 8 patients cannot exclude the possibility of SLE. dermatomyositis have not been performed. Hence, Electrocardiography (ECG) of the patient showed steroid therapy followed by other immunosuppressive results within normal limit, no conduction abnormality agents is largely empirical but widely accepted. 2,4,11,14,18 and arrhytmias were detected by ECG. In DM, clinical Clinical experience suggested that addition manifestations of heart involvement are rare, subclinical of immunosuppressive drugs to glucocorticoid is 2,8,14 manifestations are frequently reported. The EMG indicated in the majority of the patients. Many experts results of our patient suggested myositis. EMG is recommended early introduction of immunosuppressive part of diagnostic procedure. The myopathic changes treatment in severe case with poor prognosis and include myopathic motor unit potentials with or without also in order to reduce the side effect of long-term spontaneous discharge and fibrillation. However, glucocorticoid treatment such as steroid myopathies, normal EMG result does not preclude myositis. osteoporosis, cataract, aseptic necrosis, etc.7,14,15 The sensitivity of EMG changes is uncertain; therefore, Azathioprine, a purine analog that inhibits purine EMG is not recommended as an outcome measure synthesis resulting in inhibition of deoxyribonucleic during treatment.3,12 Muscle biopsy is important to acid (DNA), ribonucleic acid (RNA), and protein FRQ¿UPLQÀDPPDWRU\FKDQJHVWRGLVWLQJXLVKEHWZHHQ synthesis, may decrease proliferation of immune cell PM and IBM, to exclude other myopathies. Needle leading to lower autoimmune activity. Chloroquine PXVFOHELRSV\VKRZHG¿EHUQHFURVLVDQGUHJHQHUDWLRQ phosphate inhibits chemotaxis of eosinophils and LQDVVRFLDWLRQZLWKLQÀDPPDWRU\FHOOLQ¿OWUDWHVZLWK locomotion of neutrophils as well as impairs the lymphocytes adjacent to the blood vessels and between 12,19 PXVFOH¿EHUV1RWDEO\DSRVLWLYHPXVFOHELRSV\LV complement-dependent antigen-antibody reaction. UHTXLUHGIRUHVWDEOLVKLQJDGH¿QLWLYHGLDJQRVLVRI'0 Intravenous immunoglobulin (IV IG) has been used Dysphagia occurs in 10–73% of patients with in patients who are refractory to steroid-sparing LQÀDPPDWRU\P\RSDWKLHV6 The dysphagia associated treatment. IV IG has been shown to have positive effect with these myopathies primarily affects the skeletal on myopathy. The dose recommendation of 2 g/kg BW muscle–activated oropharyngeal phase of swallowing. should be repeated six weeks depending upon clinical It may precede weakness of the extremities or present response. Rituximab have been seen effective for DM as the sole symptom. Dysphagia is associated with and the result of large controlled ongoing studies are 2,5,8,12,18 QXWULWLRQDOGH¿FLWVDVSLUDWLRQSQHXPRQLDGHFUHDVHG being expected. quality of life, and poor prognosis.4,11,16,17 A variety of medical, rehabilitation, and interventional Oropharyngeal dysphagia was defined as WUHDWPHQWVDUHXVHGWRWUHDWLQÀDPPDWRU\P\RSDWK\ GLI¿FXOW\LQVZDOORZLQJWRJHWKHUZLWKRQHRUPRUHRI associated dysphagia. Medical treatment emphasizes the following deglutitive symptoms, including the control of disease process; whereas rehabilitation bolus hold-up, multiple swallows required to clear focuses on swallowing compensation techniques, the , deglutitive coughing and/or choking, or exercises, and diet modification. Interventional post-nasal regurgitation.17 measures include cricopharyngeal or esophageal

Volume 12, Number 1, April 2011 61 Adeputri Tanesha Idhayu, Marcellus Simadibrata, Gerie Amarendra dilation, cricopharyngeal myotomy, and botulinum Patients with dermatomyositis who have malignancy, injections of the upper esophageal sphincter. Although cardiac involvement, or pulmonary involvement some studies frequently find procedures such as and those elderly patients with dermatomyositis cricopharyngeal myotomy is beneficial, however, (> 60 years) have a poorer prognosis. The disease may the application of rehabilitation measures is far less show spontaneous remission in approximately 20% clear. Swallowing compensation techniques also have of patients. About 5% of patients have a fulminant an important role to play. Percutaneus endoscopic progressive course, with eventual death. Therefore, gastrostomy (PEG) tube insertion may be required if many patients require long-term therapy.11 swallowing remains unsafe but the highest mortality The presence of dysphagia in patients with has been described in such patients.1 myositis infers higher mortality rate. Williams Glucocorticoid therapy for our patient was initiated HWDOFRQ¿UPWKHSRRUSURJQRVLVRIWKLVDVVRFLDWLRQ with methylprednisolone at a dose of 1 mg/kg/day Despite multidisciplinary care of such cases, 31% WLPHVPJGD\ )RUWKH¿UVWIRXUWRVL[ZHHNVRI of patients in their study died of their disease within therapy, methylprednisolone was continued at 1 mg/kg/ 12 months of diagnosis and the mortality relates mainly day with ongoing assessment of the clinical response. to respiratory complications.4 After optimal therapy After that, we will taper the dose. Maintenance of of immunotherapeutic agents we expect resolution of steroid dose at 1 mg/kg per day should not persist dysphagia in patients with myositis. beyond six weeks because of the potential for the development of glucocorticoid myopathy. After four REFERENCES to six weeks at the initial dose, the steroid tapering 1. Oh TH, Brumfield KA, Hoskin TL, Stolp KA, Murray down should begin. -$%DVVIRUG-5'\VSKDJLDLQLQÀDPPDWRU\P\RSDWK\ Treatment for skin disease with sun avoidance and clinical characteristics, treatment strategies, and outcome in 62 patients. Mayo Clin Proc 2007;82:441-7. K\GUR[\FKORURTXLQHKDVEHHQEHQH¿FLDO5HKDELOLWDWLRQ 2. /XQGEUHUJ(,9HQFRYVN\-'DQL/3RO\P\RVLWLVGHUPD and physical therapy are important in management tomyositis, inflamatory diseases of muscle and other of myositis. Physical exercise now has been P\RSDWKLHV,Q%LMVPD-:%XUQHVWHQ5*-RVH3$ recommended as combination therapy together with .XHU)/HGV(XODU&RPSHQGLXPRQWKH5KHXPDWLF'LVHDVH th immunosuppressive treatment. Combining exercise 1 HG/RQGRQ%0-3XEOS with immunosuppressive therapy is safe and has clear 3. %RKDQ$3HWHU-%%RZPDQ5/3HDUVRQ&0&RPSXWHU assisted analysis of 153 patients with polymyositis and 4,5,14,18 EHQH¿FLDOHIIHFWRQPXVFOHIXQFWLRQ dermatomyositis. Medicine 1977;56:255-86. Our patient took sun protection cream and steroid 4. :LOOLDPV5%*UHKDQ0-+HUVFK0$QGUH-&RRN WRSLFDOIRUKLVVNLQ)RUSK\VLFDOWKHUDS\KHGLGH[HUFLVH ,-%LRPHFKDQLFVGLDJQRVLVDQGWUHDWPHQWRXWFRPHLQ supervised by the physician from the rehabilitation inflammatory myopathy presenting as oropharyngeal medic department. The goal of therapy was to improve dysphagia. Gut 2003;52:471-8. 5. -RVKL'0DKPRRG5:LOOLDP3.LWFKHU3'\VSKDJLD muscle strength; thereby, improving the function in secondary to dermatomyositis treated successfully with daily activities and ameliorate the extra-muscular intra venous immunoglobulin: a case report. Int Archives of manifestations. When strength improves, the serum Med [cited 2010 Aug 18]. Available from URL: http://www. CK level reduced concurrently, however the reverse intarchmed.com/content/1/1/12. is not always true. CK level is used to monitor disease 6. Robert WL. Inflammatory disease of muscle and other myopathies. In: Ruddy S, Hariss DE, Sledge B, Susgent S, activity, but the level may have poor correlation with Budd C, eds. Kelley’s Textbook of Rheumatology. 6th ed. clinical disability. Unfortunately, there is a common Philadelpia: Elsevier Saunders 2001.p.1273. tendency to “chase” or treat the CK level instead of 7. %UD\9-0L[HGFRQQHFWLYHWLVVXHGLVHDVHRYHUODSV\QGURPH the muscle weakness, a practice that has led to and undifferentiated connective tissue disease. In: West S, ed. prolonged and unnecessary use of immunosuppressive Rheumatology Secrets. 2nd ed. Philadelpia: Hanley & Belfus GUXJVDQGHUURQHRXVDVVHVVPHQWRIWKHLUHI¿FDF\5 2002.p.173-7. 8. 6WRQH+-+HOOHQ%3ROLRP\RVLWLVDQGGHUPDWRP\RVLWLV After treatment, the general condition of our patient ,Q'DYLG$:7LPRW\0&-RKQ')%HQV'0(GZDUG has become better, the patient could consume food by -%HGV2[IRUG7H[WERRNRI0HGLFLQHth ed. Oxford: RUDOURXWHDQGWKHZHDNQHVVVORZO\GLVDSSHDUHG)URP Oxford University Press 2003.p.437. laboratory results, we found that the AST has decreased 9. -HIIU\&35REHUW:/'HUPDWRP\RVLWLV&OLQLF'HUPDWRO to 75 U/L and ALT has decreased to 44 U/L. CK, LDH, 2006;24:1-5. aldolase, AST, and ALT are the muscle enzymes that 10. Dalakas MC. Polymyositis, Dermatomyositis and inclusion ERG\P\RVLWLVLQWURGXFWLRQ,Q%UDXQZDOG()DXFL$6 routinely been measured in the evaluation of myopathy. Kasper DL, Hauser SL, eds. Harrison’s Principles of Internal The prognosis of dermatomyositis depends on Medicine. 17th ed. New York: McGraw Hill 2008.p.2696-703. the severity of the myopathy, the presence of malignancy, 11. -HIU\&3'HUPDWRP\RVLWLV0HGVFDSH>FLWHG2FW@ and/or the presence of cardiopulmonary involvement. Available from URL: http://www.emedicine.medscape.com/ article/332783-overview.

62 The Indonesian Journal of Gastroenterology, Hepatology, and Digestive Endoscopy Dysphagia as an Early Presenting Symptom in Dermatomyositis

12. 6WHSKHQ-00D[LQH$3/DZUHQFH0,GLRSDWKLF,QÀDPPDWRU\ 16. Ana B, Stephen P. Polymyositis and dermatomyositis. In: PLRSDW\,Q6WHSKHQ-00D[LQH$3/DZUHQFH0HGV Clinical Medicine. 3rd ed. Philadelpia: Saunders 2003.p.260-1. Curent Medical Diagnosis and Treatment. 45th ed. New York: 17. 0LOOHU):3ROLPLRVWLVDQGGHUPDWRP\LVLWLV,Q*ROGPDQ McGraw-Hill Medical 2008.p.841-2. L, Ausiello D, eds. Cecil Textbook of Medicine. 23rd ed. 13. Kahl LE. Arthritis and rheumatologic disease. In: Green GB, Philadelpia: Elsevier Saunders 2007.p.2045-9. Harris IS, Lin GA, Moyland KC, eds. The Washington Manual 18. Ziminsky CM. Selected topics in rheumatology. In: Miller of Medical Therapeutics. 31rd ed. Lippincott: Williams & 5$VKDU%6LVVRQ6HGV-RKQV+RSNLQV,QWHUQDO0HGLFLQH Wilkins 2004.p.506-30. %RDUG5HYLHZ&HUWL¿FDWLRQDQG5HFHUWL¿FDWLRQ 14. Rubenstein D. Dermatomyositis. In: Rubenstein D, 3rd ed. Philadelpia: Mosby Elsevier 2010.p.376-87. :D\QH'%UDGOH\-HGV/HFWXUH1RWHRQ&OLQLFDO0HGLFLQH 19. Longmore M, Wilkinson L. Autoimmune connective tissue 6th ed. Oxford: Blackwell Publ 2003.p.122-39. disease. In: Longmore M, Wilkinson L, Turmezei T, Cheung 15. Hazlemen B. Musculoskeletal and connective tissue disease. KC, eds. Oxford Handbook of Clinical Medicine. 7th ed. ,Q6RXKDPL5/0R[KDP-HGV7H[WERRNRI0HGLFLQH Oxford: Oxford University Press 2008.p.538-9. 4th ed. Philadelpia: Churchill Livingstone 2004.p.1119-92.

Correspondence: Marcellus Simadibrata Division of Gastroenterology, Department of Internal Medicine Dr. Cipto Mangunkusumo General National Hospital Jl. Diponegoro No. 71 Jakarta 10430 Indonesia Phone: +62-21-3153957 Facsimile: +62-21-3142454 E-mail: [email protected]

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