Systemic LUPUS Erythematosus

dr. Rachmat Gunadi WachjudiPemerhati Lupus

LUPUS ?

LUPUSLUPUS

1.Penyakit Autoimun

2. Pembentukan berbagai autoantibodi

3. Gangguan fungsi dan kerusakan struktur jaringan organ

3. “Sembuh” dan kambuh

4. Gejala sangat beragam

Organ yang dapat dikenai ?

Sistem otot dan tulang Kulit dan rambutMata Ginjal Jantung Pembuluh darah Susunan saraf Paru-paru Komponen darah Hati

LUPUS

• Penyakit 1000 wajah• Wajah Serigala• Wajah Kupu-kupu

Siapa yang dapat mengalami LUPUS

Seberapa sering ?• 80-90% pasien lupus : wanita.

• 80% of pasien lupus berusia :

15 sampai 45 tahun.

• Lupus lebih banyak pada

Africa- Amerika dan orang Asia

Penyebab LUPUS?Penyebab LUPUS?

Faktor Pemicu dari LingkunganFaktor Pemicu dari Lingkungan

• Sinar Ultraviolet • Stres• Obat-obatan • Infeksi

• Bahan kimia• Hormon

Bercak diskoid

Kulit & mukosa

Vaskulitis Rambut rontok

Bercak malar

Peka cahaya

Sariawan berulang

Kulit & mukosa

Radang pembuluh darah

Sumbatan pembuluh darah

Penguncupan pembuluh darah

Radang sendi

Dermatological:• malar rash• discoid lesions• hair loss• oral ulcers• Raynaud’s• Nailfold erythema/crust• livedo on hands/legs• Bullous rash on legs• dermatitis on fingers

Renal:• Differing types of glomerulonephritisGI:•Gastritis/peptic ulcer due to NSAID/corticosteroids•Pancreatitis, peritonitis, and colitis: due to SLE vasculitis•Lupoid hepatitis•hepatosplenomegaly

Arthritis:•migratory and asymmetrical. Only a few joints are usually affected, especially the hands•Joint deformities

Pulmonary:• Dyspnea and restrictive LFTs•Pleurisy, pleural effusion, pneumonitis, interstitial lung disease, and pulmonary hypertension

Cardiovascular:•Pericarditis•Verrucous endocarditis => emboli•CAD from steroids

CNS:•cognitive defects, anxiety, depression, psychosis, seizures, and/or neuropathies, cerebral punctate vasculitis

Hematological:•Anemia of chronic disease•Asymptomatic leukopenia•Thrombocytopenia•lymphadenopathy

Lupus

Mendiagnosa lupusMendiagnosa lupus

LUPUS memiliki KRITERIA DIAGNOSIS berdasarkan “AMERICAN COLLEGE of RHEUMATOLOGY

1. Ruam malar2. Ruam diskoid 3. Fotosensitivitas4. Ulkus di mulut5. Artritis6. Serositis7. Gangguan ginjal 8. Gangguan Neurologi 9. Gangguan hematologi10. Gangguan Immunologi (Anti ds DNA, Anti Sm dll)11. Anti-nuclear antibody (ANA)

Kriteria ACR

Ada 4 dari 11 = SLE

Leading Causes of Death in Leading Causes of Death in SLESLE

Active lupusActive lupus

InfectionInfection

Cardiovascular diseaseCardiovascular disease

Terapi Lupus

• Mengendalikan gejala• Mengendalikan

– aktivitas sel yang autoreaktif

– pembentukan auto-antibodi

– aktivitas peradangan

Treatment of SLE

New FDA-Approved Agent – Belimumab New FDA-Approved Agent – Belimumab (Benlysta(Benlysta®®))

• Anti-BLYS humanized monoclonal antibody. Ongoing Anti-BLYS humanized monoclonal antibody. Ongoing Phase IV trials in African-American patients (multi-Phase IV trials in African-American patients (multi-center trial including UTMB)center trial including UTMB)

• Problematic indications: not for thrombocytopenia, Problematic indications: not for thrombocytopenia, CNS, or renal lupusCNS, or renal lupus

• Helpful but modest efficacyHelpful but modest efficacy

• It helps reduce steroids, prevent flares, and maintain It helps reduce steroids, prevent flares, and maintain disease remission! disease remission!

The Future - Biomarkers and Targeted TherapiesThe Future - Biomarkers and Targeted Therapies• Develop better biomarkers for flares and predictors of responseDevelop better biomarkers for flares and predictors of response

• Corticosteroid-free regimensCorticosteroid-free regimens

• Other B cell blockers, e.g., ocrelizumab, epratuzumab, TACI-Ig Other B cell blockers, e.g., ocrelizumab, epratuzumab, TACI-Ig (atacicept, an anti-BLyS/April agent). Ongoing trials (atacicept, an anti-BLyS/April agent). Ongoing trials

• Interferon alpha (IFNInterferon alpha (IFN) blockers, e.g., sifalimumab. Good ) blockers, e.g., sifalimumab. Good promising data. Ongoing trialspromising data. Ongoing trials

• Anti-C5: humanized monoclonal Ab, especially for APS, ongoing Anti-C5: humanized monoclonal Ab, especially for APS, ongoing trials, including UTMB trials, including UTMB

• Interferon gamma (IFNInterferon gamma (IFNγγ)) blockers: for renal lupus. Ongoing trials blockers: for renal lupus. Ongoing trials • Petri M, et al. Sifalimumab, a human anti-IFN alpha antibody in SLE. A & R 65; 2013: 1011-21Petri M, et al. Sifalimumab, a human anti-IFN alpha antibody in SLE. A & R 65; 2013: 1011-21

Every patient with lupus should be on vitamin Every patient with lupus should be on vitamin D and hydroxychloroquine (HCQ)!D and hydroxychloroquine (HCQ)!

• A 20-ng/ml increase in the 25 (OH) D level was associated with a A 20-ng/ml increase in the 25 (OH) D level was associated with a 21% decrease in the odds of having a high disease activity score 21% decrease in the odds of having a high disease activity score

• Fifteen (15%) decrease in the odds of having clinically important Fifteen (15%) decrease in the odds of having clinically important proteinuria proteinuria

• There was no evidence of additional benefit of 25 (OH) D There was no evidence of additional benefit of 25 (OH) D beyond a level of 40 ng/mlbeyond a level of 40 ng/ml

Petri M, et al. A & R 2013; 65: 1865–1871Petri M, et al. A & R 2013; 65: 1865–1871Willis R, Jajoria P, Harper BE, Gonzalez EB, Petri M, Akhter E, Fang H, Pierangeli SS, Willis R, Jajoria P, Harper BE, Gonzalez EB, Petri M, Akhter E, Fang H, Pierangeli SS, Abstract Abstract 691, ACR Annual meeting, Washington, D.C, Nov 2012, S296.691, ACR Annual meeting, Washington, D.C, Nov 2012, S296.

Dampak LupusPenyakit menahun

Beban biaya Terus menerus makan obat efek sampingKeputus-asaan / bosan berobat mencari pengobatan di luar medis

Tekanan dari institusi dan lingkungan kerja/pendidikan

berhenti bekerja/sekolahDisangka penyakit menular dijauhi

Efek samping obat

• Infeksi• Metabolisme• Osteoporosis• Kosmetik

Bisakah odapus punya keturunanan ?

• Lupus terkendali• Perlu penilaian di awal• Bisa “flare”• Lupus bayi

Berapa lama lagi ….. ?Umur harapan hidup 10 tahun yad 85 %

Hidup dengan LUPUS ?Dapat hidup normalSesuaikan aktifitas dan pola hidup dengan kondisi Hindari faktor pencetus

Pajanan sinar matahari yang lama & lampu fluoresensi

Obat-obat yang meningkatkan fotosensitifitas

Tertular penyakit (kerumunan, tempat ramai)

Tekun dan sabar menjalani pengobatan secara teratur dan konsultasi secara aktif dengan dokterBerupaya mengenal dan memahami lupus dan mengikuti perkembangannya

Hidup dengan LUPUS ? Gangguan mental emosional memerlukan

dokter yang mampu / bersedia / punya waktu untuk memberikan edukasidukungan keluarga, teman dan sesama odapus

Edukasi LUPUS• Hal yang sangat

diperlukan

Komunikasi yang baik dokter-pasien

Kelompok edukasi/support Group

Waspadai Jika ……

‘Growing pains’ pada ABGMigraine-like headache pada ABG‘Glandular fever’ pada ABGDigigit nyamuk reaksinya berlebihanKeguguran berulangBanyak keluhan menjelang menstruasiAlergi sulfa

Waspadai Jika ……

Agorafobia atau klaustrofobiaTendinitis fleksor pada tanganAda keluarga dengan penyakit autoimunMata sering sepetCRP normal tapi LED tinggiLimfopeniaC4 borderline rendah

Segera hubungi dokter jika mengalami :

Demam tinggiSakit kepala hebatDarah pada urinNyeri dadaSesak nafasBengkak tungkai / kakiKelemahan anggota gerakNyeri perut yang hebatNyeri sendi yang tidak biasaGangguan penglihatan

Kepada Siapa Bertanya ?Kepada Siapa Bertanya ?

• Dokter umum• Spesialis Penyakit

Dalam• Reumatologist• Dermatologist• Nephrologist (ahli

ginjal)• Spesialis lain (mata,

THT, kulit )

• Yayasan Lupus Indonesia

• Syamsi Dhuha Foundation

• Support Grup lainnya

Hatur Nuhun