Bullous Esophagitis with Severe Stricturing Disease, a Case Study of a Rare Systemic Lupus Erythematosus Manifestation
Bibliographic record
Abstract
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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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.002 |
| Meta-epidemiology (narrow) | 0.002 | 0.001 |
| Meta-epidemiology (broad) | 0.001 | 0.001 |
| Bibliometrics | 0.002 | 0.002 |
| Science and technology studies | 0.002 | 0.001 |
| Scholarly communication | 0.002 | 0.002 |
| Open science | 0.001 | 0.002 |
| Research integrity | 0.003 | 0.002 |
| Insufficient payload (model declined to judge) | 0.002 | 0.001 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".