Type II Achalasia as the Initial Presentation of Systemic Sclerosis
Bibliographic record
Abstract
A 62-year-old female with a history of coronary artery disease and longstanding Raynaud’s syndrome was referred to us for an assessment of noncardiac chest pain, dysphagia and nocturnal reflux. Esophagogastroduodenoscopy was unremarkable with no evidence of stricture or mass. High-resolution esophageal manometry showed Type II achalasia (Figure 1). Ambulatory 24-hour pH study showed no significant acid reflux. A year later, she presented with progression of Raynaud’s. Physical examination demonstrated sclerodactyly, digital capillary dropout and chest wall telangiectasias. Bloodwork was significant for positive antinuclear antibody (1:80) and anticentromere antibody (1:320). She was subsequently diagnosed with limited cutaneous systemic sclerosis. Follow-up manometry (3 years later) showed classic ‘scleroderma esophagus’ (Figure 2). Clouse plot demonstrating Type II achalasia (integrated residual pressure = 19.2, normal < 15 mmHg; panesophageal pressurization with 70% of swallows). Clouse plot demonstrating hypotensive lower esophageal sphincter, and absent peristalsis, consistent with ‘scleroderma esophagus’. Vasculopathy leading to smooth muscle atrophy and fibrotic replacement is thought to be responsible for the classic finding of ‘scleroderma esophagus’, characterized by hypotensive lower esophageal sphincter (LES) pressure and aperistalsis in the distal esophagus (1). However, an achalasia-like syndrome with incomplete LES relaxation has been reported in systemic sclerosis (SSc) patients. Park et al. first described aperistalsis and incomplete LES relaxation on conventional manometry in four of seven patients with known SSc (2). The finding of esophageal dilation and delayed esophageal emptying on barium study, associated with manometric evidence of aperistalsis with normal LES relaxation, was documented to precede autoantibody formation and skin changes in a patient who developed SSc; follow-up manometry demonstrated absent motor activity in the smooth muscle esophagus with absent LES tone (3). The pathophysiology of this finding is unclear but may relate to the presence of antimyenteric neuronal antibodies. Antimyenteric neuronal antibodies have been detected in the sera of SSc patients and correlate with the presence of Raynaud’s phenomenon (4). Autoantibodies to the myenteric plexus have also been detected in achalasia patients, and may be implicated in the decreased interstitial cells of Cajal and nitric oxide synthase containing neurons seen on histopathologic examination (5). Our case is the first to document on high-resolution manometry the conversion of type II achalasia features to scleroderma esophagus findings. This may yield insight into the pathophysiology of esophageal disease in SSc, which remains poorly understood. Grant support: none. Disclosures: none.
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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 | 0.003 |
| Meta-epidemiology (narrow) | 0.001 | 0.001 |
| Meta-epidemiology (broad) | 0.001 | 0.001 |
| Bibliometrics | 0.002 | 0.002 |
| Science and technology studies | 0.001 | 0.001 |
| Scholarly communication | 0.002 | 0.001 |
| Open science | 0.001 | 0.001 |
| Research integrity | 0.003 | 0.002 |
| Insufficient payload (model declined to judge) | 0.003 | 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".