What is the management approach for a patient with Stevens-Johnson Syndrome (SJS) and transaminitis?

Management of Stevens-Johnson Syndrome with Transaminitis

Immediate Actions

Discontinue all suspected culprit drugs immediately and transfer the patient to a specialized burn unit or ICU with multidisciplinary expertise, particularly when body surface area involvement exceeds 10%, while initiating comprehensive supportive care as the cornerstone of management. 1, 2, 3

• Calculate SCORTEN within the first 24 hours to predict mortality risk and guide intensity of care 1, 2, 3
• Transfer without delay to centers with multidisciplinary teams including dermatology, intensive care, ophthalmology, and hepatology given the transaminitis 1, 2
• Early transfer reduces mortality; delays in specialized care adversely affect outcomes 1

Hepatic Monitoring and Management

Monitor liver function tests serially (transaminases, bilirubin, alkaline phosphatase, γ-glutamyl transferase) as cholestatic hepatitis can develop during the acute phase of SJS and typically resolves with supportive care. 4

• Transaminitis in SJS may represent drug-induced hepatotoxicity from the culprit medication or evolve into cholestatic hepatitis 4
• Adequate supportive care alone typically leads to normalization of liver tests within weeks as skin lesions resolve 4
• Avoid hepatotoxic medications during the acute phase 4
• Consider hepatology consultation if transaminases continue rising or if signs of hepatic dysfunction develop 1

Specialized Care Environment

• Barrier-nurse in a temperature-controlled room (25-28°C) on a pressure-relieving mattress with humidity control 1, 2, 3
• This prevents hypothermia and reduces infection risk 1

Fluid Management

• Provide careful fluid resuscitation to prevent end-organ hypoperfusion (including hepatic hypoperfusion) while avoiding overaggressive replacement that leads to pulmonary, cutaneous, and intestinal edema 1, 2, 3
• Monitor fluid balance with urinary catheterization when indicated, regular assessment of vital signs, urine output, and electrolytes 1, 2
• Use hemodynamic parameters to guide replacement volumes 2

Wound Care Protocol

• Handle skin with extreme gentleness to minimize shearing forces that cause further epidermal detachment 1, 2, 3
• Regularly cleanse wounds by gently irrigating with warmed sterile water, saline, or chlorhexidine (1/5000) 1, 2
• Apply greasy emollient (50% white soft paraffin with 50% liquid paraffin) over entire epidermis including denuded areas every 2-4 hours 1, 2, 3
• Leave detached epidermis in situ to act as biological dressing 1, 2
• Apply nonadherent dressings (Mepitel™ or Telfa™) to denuded dermis with secondary foam or burn dressings to collect exudate 1, 2

Infection Prevention and Management

Do NOT administer prophylactic systemic antibiotics as this increases skin colonization with resistant organisms, particularly Candida albicans. 1, 2, 3, 5

• Take swabs for bacterial and candidal culture from three lesional areas on alternate days 1, 2
• Monitor for clinical signs of infection: confusion, hypotension, reduced urine output, reduced oxygen saturation, rising C-reactive protein, and neutrophilia 1, 2
• Institute targeted antimicrobial therapy ONLY when clinical signs of infection appear 1, 2, 3
• Monoculture of organisms on culture swabs from multiple sites indicates increased likelihood of invasive infection 2, 3
• Septicemia is the most common cause of death in SJS/TEN 6

Critical Mucosal Management

Ophthalmological Care

Arrange ophthalmological examination within 24 hours of diagnosis with daily reviews throughout the acute illness to prevent permanent visual impairment. 1, 2, 3, 5

• Apply preservative-free lubricant eye drops every 2 hours throughout the acute illness 1, 2, 3
• Perform daily ocular hygiene by an ophthalmologist or ophthalmically trained nurse to remove inflammatory debris and break down conjunctival adhesions 1, 3
• Use topical antibiotics when corneal fluorescein staining or ulceration is present 1
• Consider topical corticosteroids for ocular inflammation under ophthalmologist supervision 1, 3
• Consider amniotic membrane transplantation in the acute phase for significantly better visual outcomes 1

Oral Care

• Perform daily oral review during the acute illness 1, 2
• Apply white soft paraffin ointment to the lips every 2 hours throughout the acute illness 1, 2
• Use anti-inflammatory oral rinse containing benzydamine hydrochloride every 3 hours, particularly before eating 1
• Apply antiseptic oral rinse twice daily to reduce bacterial colonization 1
• Treat candidal infection with nystatin oral suspension 100,000 units four times daily for 1 week, or miconazole 1

Urogenital Care

• Perform regular examination of urogenital tract during acute illness 1
• Apply white soft paraffin ointment to urogenital skin and mucosae every 4 hours 1
• Consider vaginal dilators or tampons wrapped in Mepitel to prevent vaginal synechiae formation 1

Nutrition Support

• Provide continuous enteral nutrition throughout the acute phase 1, 2
• Deliver 20-25 kcal/kg daily during the catabolic phase 1, 2
• Increase to 25-30 kcal/kg daily during the anabolic recovery phase 1, 2
• Consider nasogastric feeding when oral intake is precluded by buccal mucositis 1

Pain Management

• Use validated pain assessment tools at least once daily 1, 2
• Provide adequate background simple analgesia with additional opioid analgesia for breakthrough pain 1, 2
• Consider patient-controlled analgesia where appropriate 2
• Consider sedation or general anesthesia for patient handling, repositioning, and dressing changes 2

Systemic Immunomodulatory Therapy

Consider IV methylprednisolone 0.5-1 mg/kg if started within 72 hours of onset, converting to oral corticosteroids on response with tapering over at least 4 weeks. 1, 3

• Multiple studies show benefit when corticosteroids are initiated early (within 72 hours) 1, 3
• Alternative option: Cyclosporine 3 mg/kg daily for 10 days, tapered over 1 month, which has shown reduced mortality compared to predicted rates 1, 3
• IVIg has equivocal evidence with pooled analysis showing no mortality benefit (OR 1.00,95% CI 0.58-1.75) 1
• Thalidomide should be AVOIDED as it was associated with excess deaths in one randomized trial 1

Caution with Systemic Therapy in Hepatic Dysfunction

• Given the transaminitis, monitor liver function closely if initiating systemic corticosteroids or cyclosporine 4
• Both agents can affect hepatic function, though the benefit in reducing SJS/TEN mortality typically outweighs this risk when started early 1, 3

Additional Supportive Measures

• Administer low molecular weight heparin as prophylactic anticoagulation for immobile patients 1
• Provide proton pump inhibitor if enteral nutrition cannot be established 1
• Consider recombinant human G-CSF for neutropenic patients 1

Airway Management

• Respiratory symptoms and hypoxemia on admission should prompt urgent discussion with an intensivist and rapid transfer to ICU or burn center 1
• Perform fibreoptic bronchoscopy to identify bronchial involvement, evaluate prognosis, investigate pneumonitis, and mechanically remove sloughed bronchial epithelium 1

Multidisciplinary Team Coordination

Coordinate care through a team led by a dermatologist or specialist in skin failure, including intensive care physicians, hepatologist (given transaminitis), ophthalmologists, and specialist skincare nurses as core team members. 1, 2, 3, 5

• Additional specialists may include respiratory medicine, gastroenterology, gynecology, urology, oral medicine, microbiology, pain team, dietetics, physiotherapy, and pharmacy 1, 2

Discharge Planning and Long-Term Follow-Up

• Provide written information about the culprit drug(s) to avoid and any potentially cross-reactive medications 1, 2, 3
• Encourage patients to wear a MedicAlert bracelet bearing the name of the culprit drug 1, 2, 3
• Document drug allergy in the patient's medical records and inform all healthcare providers involved in their care 1, 2, 3
• Report the adverse drug reaction to pharmacovigilance authorities 1, 2, 3
• Arrange follow-up with dermatology and ophthalmology within weeks of discharge 2
• Inform patients about potential fatigue and lethargy for several weeks following discharge and the need for convalescence 1
• Ensure hepatology follow-up if transaminitis persists at discharge 4

Critical Pitfalls to Avoid

• Delayed recognition and transfer to specialized care significantly increases mortality risk 1, 2, 3
• Failure to involve ophthalmology within 24 hours leads to permanent visual impairment 1, 3, 5
• Indiscriminate use of prophylactic antibiotics increases resistant organism colonization 1, 2, 3, 5
• Overaggressive fluid resuscitation causes pulmonary, cutaneous, and intestinal edema 1, 2, 3
• Continued use of the culprit medication will worsen the condition and increase mortality 1
• Neglecting serial liver function monitoring may miss progression to cholestatic hepatitis 4