A rare case of Plummer–Vinson syndrome in a young female

We report the case of a 32-year-old female who presented with a history of progressive painless dysphagia for both solids and liquids, generalised weakness and easy fatiguability for the preceding 2 years. She was diagnosed to have hypothyroidism 2 years ago and was on regular levothyroxine supplementation therapy. General physical examination revealed conjunctival pallor and koilonychia. Systemic examination was unremarkable. Laboratory investigations revealed low haemoglobin (8.6 g/dL); total leucocyte count, platelet count and liver function tests were within normal limits. Peripheral smear examination revealed microcytic hypochromic red blood cells, depleted iron stores (serum iron 14 μg/dL, ferritin 2.8 ng/mL, total iron-binding capacity 349 μg/dL, reticulocyte count 7% and transferrin saturation 4%), suggestive of iron-deficiency anaemia (IDA). Upper gastrointestinal endoscopy revealed a cricopharyngeal stricture (20 cm from central incisors) (Figure 1). X-ray barium swallow study showed oesophageal webs (Figure 2). The patient was diagnosed to have Plummer–Vinson syndrome (PVS). The patient was treated with intravenous iron therapy with ferric carboxymaltose in two 1 g doses and was subsequently discharged with oral iron supplementation. The patient is doing well on follow-up at 3 months.Figure 1: Upper GI endoscopy showing cricopharyngeal stricture (arrow)Figure 2: X-ray barium swallow study showing oesophageal web (arrow)PVS is characterised by the classical triad of post-cricoid dysphagia, IDA and upper oesophageal webs.[1] Exact data on the incidence and prevalence of PVS are lacking; it was once common among middle-aged Northern European women in the early 20th century.[2] At present, it is extremely rare, with only isolated case reports documented in recent literature.[3] Pathogenesis of PVS is unclear, but iron deficiency is considered the key aetiological factor, as it is part of the classic triad with dysphagia and oesophageal webs, and both dysphagia and impaired oesophageal motility have been shown to improve with iron supplementation.[4] Other aetiologic factors, including malnutrition, genetic predisposition or even autoimmune processes, have been proposed.[5] Disorders associated with PVS include thyroid disease and coeliac disease, which increases the possibility of immune dysregulation.[5] The first step in the management of PVS is to clarify the cause of iron deficiency in order to exclude active haemorrhage, malignancy or coeliac disease. PVS can be treated effectively with iron supplementation, which alone has been shown to resolve dysphagia in many cases.[6] For patients with significant oesophageal obstruction or persistent symptoms, mechanical dilation is performed using bougies or balloon dilators, multiple times if required.[7] Early diagnosis and treatment can improve dysphagia and prevent future complications, including the risk of upper oesophagus cancer. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest The authors are faculty members/residents of Sri Venkateswara Institute of Medical sciences, Tirupati, of which Journal of Clinical and Scientific Research is the official Publication. The article was subject to the journal’s standard procedures, with peer review handled independently of these faculty and their research groups. Use of artificial intelligence (AI)-assisted technology for manuscript preparation The authors confirm that there was no use of artificial intelligence (AI)-assisted technology for assisting in the preparation of the manuscript and no images were manipulated using AI.