Patofisiologi kasus skleroderma pada disfagia esofagus

Abstract

Latar belakang: Disgafia adalah kesulitan menelan yang dapat disebabkan oleh kelainan kongenitalatau kelainan sistemik seperti skleroderma. Skleroderma merupakan penyakit autoimun kronis denganinsidens yang jarang yaitu 20:1.000.000. Skleroderma akan menyebabkan atrofi otot polos dan fibrosis padaesofagus sehingga menyebabkan Barrett’s esophagus, striktur, bahkan keganasan.

Tujuan: Mengetahuidan memahami patofisiologi disfagia fase esofagus yang disebabkan oleh skleroderma sehingga dapatmemberi tata laksana yang optimal.

Kasus: Perempuan berusia 31 tahun dengan disfagia fase esofaguse.c. skleroderma. Skleroderma menyebabkan atrofi dan fibrosis otot polos yang menimbulkan dismotilitasesofagus sehingga gerakan peristaltik hilang secara progresif, sfingter esofagus inferior melemah,pengosongan esofagus tertunda, dan terjadi refluks gastroesofagus.

Penatalaksanaan: Terapi suportif:Ringer Laktat (RL) 20 tpm, metil-prednisolon 125 mg 1/3-0-0, ranitidin 50 mg 1-0-1, omeprazole 20mg/12 jam, chlorpheniramin maleat (CTM) 4 mg/8 jam, soft U derm lotion topical setiap 12 jam. Terapikonservatif: pengaturan diet, berbaring dengan kepala ditinggikan, obat antasida, agen prokinetik,dan antisekretorik.

Kesimpulan: Disfagia fase esofagus dapat disebabkan oleh skleroderma, denganmemahami patofisiologinya maka akan dapat memberikan tatalaksana yang tepat.

Kata kunci: Skleroderma, disfagia esofagus, patofisiologi

ABSTRACT

Background: Disphagia is difficulty of swallowing which could be caused by congenitalabnormalities or systemic disorders such as scleroderma. Scleroderma is a rare chronic autoimmunedisease with incidence of 20:1.000,000. Scleroderma causes atrophy of smooth muscles and fibrosis ofthe esophagus, that leading to Barrett’s esophagus, strictures, or even malignancy.

Purpose: Knowingand understanding the pathophysiology of the esophageal phase dysphagia caused by scleroderma,as to provide optimal management.

Case: We reported a 31 years old female, with esophageal phasedysphagia that caused by scleroderma. Scleroderma causes smooth muscles atrophy and fibrosis thatcaused esophageal dysmotility and decrease peristaltic progressively, weakening of inferior esophagealsphincter, delayed emptying of the esophagus, and gastroesophageal reflux.

Management: Supportivetherapy: Ringer Lactate (RL) 20 drops/minute, methylprednisolone 125 mg 1/3-0-0, ranitidin 50 mg 1-0-1,omeprazole 20 mg/12 hrs, chlorpheniramine maleat (CTM) 4 mg/8 hrs, soft U-derm topical lotion every12 hrs. Conservative therapy: Diet regulation, laying down with elevated head, antacid, prokinetic, andantisecretory.

Conclusion: Esophageal phase dysphagia can be caused by scleroderma. Understandingthe pathophysiology will lead to a proper management.

Keywords: Scleroderma, dysphagia esophageal, pathophysiology

Alamat korespondensi : Nancy Liwikasari. Email: cy_nancy16@yahoo.com