Bullous Esophagitis with Severe Stricturing Disease, a Case Study of a Rare Systemic Lupus Erythematosus Manifestation
Notice bibliographique
Résumé
Background While Systemic Lupus Erythematosus (SLE) is a multisystemic disease with many manifestations, the esophagus is rarely involved. When there is esophageal involvement, it is typically reflux, esophageal dysmotility, or esophagitis secondary to medications or infection,[1] with 1 report of esophageal sloughing associated with bullous SLE.[2] Here, we present a case of a 22-year-old woman with established SLE, presenting with a recurrent esophageal stricture secondary to bullous disease. Her previous lupus manifestations included bullous skin disease, desquamative gingivitis, mild leukopenia and anemia, oronasal ulcers, and mild proteinuria (presumed lupus membranous nephropathy). Case The patient was initially seen as an outpatient with an esophageal stricture, thought then to be related to reflux from her SLE and potential esophageal dysmotility. Over several months she developed severe dysphagia causing weight loss and subsequently presented to the ED with an impacted food bolus requiring endoscopic extraction and admission for sequential dilatation. On endoscopy, she had significant abnormalities of the proximal esophagus with a normal distal esophagus, stomach, and duodenum: not consistent with reflux-induced disease. Previous biopsy demonstrated non-specific active esophagitis; biopsy on this admission demonstrated positive direct immunofluorescence with granular basement membrane zone immunoreactivity for immunoglobulins IgG and IgA, and weak immunoreactivity for IgM and C3, characteristics of immune complex deposition in SLE.[3] The differential for such bullous disease includes bullous pemphigoid, dermatitis herpetiformis, and epidermolysis bullosa acquisita. However, in this case the established SLE, granular deposition, and immune complex profile strongly suggest bullous lupus disease. The patient had previously been managed with mycophenolate mofetil 500 mg BID (due to intolerance to hydroxychloroquine); this was escalated to 1000 mg BID, and she was started on moderate dose prednisolone (approx. 0.5 mg/kg, utilizing a liquid formulation given her severe dysphagia). To date the patient has improved and is now tapering her steroids, with consideration for escalation to biologic therapy with anifrolumab. Other treatment options include dapsone: commonly used for bullous skin disease. Conclusion Bullous skin disease is an unusual lupus manifestation, and this patient’s esophageal bullous disease is an exceedingly uncommon complication. This case highlights the importance of pathology in delineating disease manifestations. While esophagitis from reflux or medications remains drastically more common, this patient benefited from having her rare complication properly identified. To that end this case also demonstrates the importance of therapeutic collaboration with mechanical intervention from surgical colleagues as well as escalated immunosuppressive therapy to target her underlying disease. [1.] Ebert E. J Clin Gastroenterol 2011;45:436-41. [2.] Yogarajah M. Case Rep Rheumatol 2015;1:930683. [3.] Contestable J. Am J Clin Dermatol 2014;15:517-24.
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Comment cette classification a été obtenuedéplier
Prédiction distillée sur la base complète
Imitation des enseignantsNi prévalence calibrée, ni vérité terrain. Validation humaine à venir. Apprise à partir de 10 348 étiquettes directes de Codex et de 10 348 étiquettes directes de Gemma. Le mode candidate est l'union des têtes enseignantes seuillées; le consensus est leur intersection. Ces sorties portent le statut machine_predicted_unvalidated et ne sont ni des étiquettes humaines ni des étiquettes directes de modèles de pointe.
Scores Codex et Gemma par catégorie
| Catégorie | Codex | Gemma |
|---|---|---|
| Métarecherche | 0,000 | 0,000 |
| Méta-épidémiologie (sens strict) | 0,000 | 0,000 |
| Méta-épidémiologie (sens large) | 0,001 | 0,000 |
| Bibliométrie | 0,000 | 0,000 |
| Études des sciences et des technologies | 0,000 | 0,000 |
| Communication savante | 0,000 | 0,000 |
| Science ouverte | 0,000 | 0,000 |
| Intégrité de la recherche | 0,000 | 0,000 |
| Charge utile insuffisante (le modèle a refusé de juger) | 0,000 | 0,000 |
Scores machine (provisoires)
Les deux têtes enseignantes du modèle étudiant, lues sur ce travail. Un score ordonne la base pour la relecture; il n'affirme jamais une catégorie, et le statut de validation accompagne chaque rangée tel quel.
Scores de référence d'un modèle non mature (critères de maturité non atteints, 7 itérations). Un score ordonne; il n'affirme jamais une catégorie.
score_only:v0-immature-baseline · tel quel depuis la passe de notation : score_only signifie que le nombre peut ordonner les travaux, et qu'aucune étiquette de catégorie n'en découleClassification
machine, non validéePrédiction automatique; un appel candidat d’une seule tête enseignante, pas un consensus.
Le détail, modèle par modèle et score par score, se trouve en fin de page sous « Comment cette classification a été obtenue ».