ASKEP GADAR SINDROM STEVEN JHONSON

Nursiswati, MKep., Sp. KMB

• Sindrom Steven-Johnson (SSJ) merupakan suatu kumpulan gejala klinis erupsi mukokutaneus yang ditandai oleh trias kelainan pada kulit vesikulobulosa, mukosa orifisium serta mata disertai gejala umum berat.

• Sinonimnya antara lain : sindrom de Friessinger-Rendu, eritema eksudativum multiform mayor, eritema poliform bulosa, sindrom muko-kutaneo-okular, dermatostomatitis, Toxic epidermal necrolysis (TEN) dll.

• While minor presentations may occur, significant involvement of oral, nasal, eye, vaginal, urethral, GI, and lower respiratory tract mucous membranes may develop in the course of the illness. GI and respiratory involvement may progress to necrosis. SJS is a serious systemic disorder with the potential for severe morbidity and even death.

• Pathophysiology: SJS adl kelainan immune-complex yg dimediasi hypersensitivity yg dpt menyebabk o/ berbagai obat-obatan, infeksi viral, dan malignancy.

• Cocaine recently has been added to the list of drugs capable of producing the syndrome. In up to half of cases, no specific etiology has been identified.

• Patogenesis SSJ sampai saat ini belum jelas walaupun sering dihubungkan dengan reaksi hipersensitivitas tipe III (reaksi kompleks imun) yang disebabkan oleh kompleks soluble dari antigen atau metabolitnya dengan antibodi IgM dan IgG dan reaksi hipersensitivitas lambat (delayed-type hypersensitivity reactions, tipe IV) adalah reaksi yang dimediasi oleh limfosit T yang spesifik.

• History: Typically, the disease process begins with a nonspecific upper respiratory tract infection.This usually is part of a 1- to 14-day prodrome during which fever, sore throat, chills, headache, and malaise may be present.Vomiting and diarrhea occasionally are noted as part of the prodrome.Mucocutaneous lesions develop abruptly. Clusters of outbreaks last from 2-4 weeks. The lesions typically are nonpruritic.A history of fever or localized worsening should suggest a superimposed infection; however, fever has been reported to occur in up to 85% of cases.

• Pada kasus yang tidak berat, prognosisnya baik, dan penyembuhan terjadi dalam waktu 2-3 minggu. Kematian berkisar antara 5-15% pada kasus berat dengan berbagai komplikasi atau pengobatan terlambat dan tidak memadai. Prognosis lebih berat bila terjadi purpura yang lebih luas. Kematian biasanya disebabkan oleh gangguan keseimbangan cairan dan elektrolit, bronkopneumonia, serta sepsis.

ETIOLOGI• The 4 etiologic categories are (1) infectious, (2) drug-

induced, (3) malignancy-related, and (4) idiopathic.Infectious diseases that have been reported include herpes simplex virus (HSV), influenza, mumps, cat-scratch fever, mycoplasmal infection, lymphogranuloma venereum (LGV), histoplasmosis, and cholera.In children, Epstein-Barr virus and enteroviruses have been identified.

• Drug etiologies include penicillins, sulfas, phenytoin (and related anticonvulsants), carbamazepine, and barbiturates. In late 2002, the US Food and Drug Administration (FDA) and the manufacturer Pharmacia noted that SJS had been reported in patients taking the cyclooxygenase-2 (COX-2) inhibitor valdecoxib.

• Various carcinomas and lymphomas have been associated.

• SJS is idiopathic in 25-50% of cases.

GEJALA KLINIK/Symptom

• Gejala prodromal berkisar antara 1-14 hari berupa demam, malaise, batuk, sakit menelan, nyeri dada, muntah, pegal otot dan atralgia yang sangat bervariasi dalam derajat berat dan kombinasi gejala tersebut.

• Involvement of oral and/or mucous membranes may be severe enough that patients may not be able to eat or drink.Patients with genitourinary involvement may complain of dysuria or an inability to void.A history of a previous outbreak of SJS or of erythema multiforme may be elicited. Recurrences may occur if the responsible agent is not eliminated or if the patient is reexposed.

• Typical symptoms are as follows:Cough productive of a thick purulent sputumHeadacheMalaiseArthralgia

• Physical: The rash can begin as macules that develop into papules, vesicles, bullae, urticarial plaques, or confluent erythema.The center of these lesions may be vesicular, purpuric, or necrotic.The typical lesion has the appearance of a target. The target is considered pathognomonic.

• Lesions may become bullous and later rupture, leaving denuded skin. The skin becomes susceptible to secondary infection.Urticarial lesions typically are not pruritic.Infection may be responsible for the scarring associated with morbidity.Although lesions may occur anywhere, the palms, soles, dorsum of hands, and extensor surfaces are most commonly affected.The rash may be confined to any one area of the body, most often the trunk.

• Mucosal involvement may include erythema, edema, sloughing, blistering, ulceration, and necrosis.The following signs may be noted on examination:FeverOrthostasisTachycardiaHypotensionAltered level of consciousnessEpistaxisConjunctivitisCorneal ulcerationsErosive vulvovaginitis or balanitisSeizures Coma

DIAGNOSTIC TEST• A complete blood count (CBC) may reveal a normal white blood cell (WBC) count

or a nonspecific leukocytosis. A severely elevated WBC count indicates the possibility of a superimposed bacterial infection.Determine renal function and evaluate urine for blood.Electrolytes and other chemistries may be needed to help manage related problems.Cultures of blood, urine, and wounds are indicated when an infection is clinically suspected.

•Kadar IgG dan IgM dapat meninggi, C3 dan C4 normal atau sedikit menurun dan dapat dideteksi adanya kompleks imun beredar. Biopsi kulit direncanakan bila lesi klasik tak ada. Imunoflurosesensi direk bisa membantu diagnosa kasus-kasus atipik.

• Imaging Studies: Chest radiograph may indicate the existence of a pneumonitis when clinically suspected. Otherwise, routine plain films are not indicated.

Other Tests: Skin biopsy is the definitive diagnostic study but is not an ED procedure.Skin biopsy demonstrates that the bullae are subepidermal.Epidermal cell necrosis may be noted.Perivascular areas are infiltrated with lymphocytes.

NURSING DIAGNOSES

• ALTERED RESPIRATORY• FLUID VOLUME DIFICITS• IMPARED TISSUE INTEGRITY• PAIN• ALTERED BODY TEMPERATURE

NURSING MANAGEMENT

• MAINTAINING VENTILATION• MANAGING POTENTIAL COMPLICATIONS:

SEPSIS• MAINTAINING FLUID BALANCE• PREVENTING HYPOTHERMIA• RELIEVING PAIN

SYSTEMIC INFLAMMATORY RESPONSE SYNDROME

• ADL PASIEN YG MEMILIKI 2 ATAU LEBIH KRITERIA SBB :

1. SUHU > 38 C ATAU < 36 C2. HR > 90 X/MNT3. RR> 20 X/MNT ATAU PaCO2 <32 mmHg4. Hit lekosit > 12.000/mm3 atau > 10 % sel imaturKONFERENSI TERBARU→ PROCALCITONIN(PCT)

DAN C-REACTIVE PROTEIN(CRP)

• SEPSIS ADL SIRS DITAMBAH TEMPAT INFEKSI YG DIKET (DITENT DG BIAKAN POSITIF THD ORGANISME DR TEMPAT TSB)

• MESKIPUN SEPSIS, SIRS DAN SYOK SEPTIK BIASANYA BERHUB DG INF BAKTERI, TDK HRS TERDPT BAKTERIMIA. HAL INI DIKARENAKAN DL DARAH KEMUNGKINAN TERDPT ENDO MAUPUN EKSOTOKSEMIA, SEDANGKAN BAKTERI BERADA DI JARINGAN.

ETIOLOGI-PATOFIS• PENYEBAB TERTINGGI SEPSIS ADL BAKTERI GRAM (-)

DG PROSENTASE 60-70 % KASUS, YG MHASILKAN BBG PRODUK DPT MENSTIMULASI SEL IMUN.

• SEL TSB AKN TERPACU U MELEPASKAN MEDIATOR INFLAMASI.

• PRODUK YG BPERAN PENTING ADL LIPOPOLISAKARIDA ATAU ENDOTOKSIN GLIKOPROTEIN KOMPLEKS MRP KOMPONEN UTAMA MEMBRAN TERLUAR DR BAKTERI GRAM (-)

• LIPOPOLISAKARIDA/LPS MERANGSANG PERADANGAN JARINGAN, DEMAM DAN SYOK

• LPS DI DL DARAH AKN BERIKATAN DG PROTEIN DRH MBTK LIPOPOLISACCHARIDE BINDING PROTEIN/LBP.

• LBP DPT MENGAKTIFKN ST IMUN SELULER DAN HUMORAL, YG DPT MENIMBULK PERKEMB GEJALA SEPTIKEMIA.

PENGKAJIAN

• ABC• LEUKOSITOSIS, TROMBOSITOPENIA, D-dimer• Asidosis metabolik• ARDS• DIC• GGA• PERDARAHAN USUS

• GAGAL HATI• DISFUNGSI SSP• GAGAL JANTUNG

INTERVENSI

• STABILISASI ABC : INTUBASI, VENTILASI MEKANIS, KRISTALOID/KOLOID

• INOTROP/VASOPRESOR (DOPAMIN, DOBU, EPINEFRIN, NOREPINEFRIN)

• CVP• AB: KARBAPENEM,CEFTRIAXONE, SEFEPIM,

AMINOGLIKOSID, QUINOLON.

THANKS