- Evaluate a patient with odynophagia
A 45 yo M with asthma presents with inability to tolerate food and painful swallowing x 2 weeks. He first noticed symptoms ~ 4 months ago but they were mild and intermittent. Over the past 2 weeks, he’s lost 15 lbs and has been unable to tolerate both solids and liquids. He has severe midline chest pain with swallowing and states that “food tastes funny”.
He has taken some ibuprofen initially but now cannot swallow any pills. He otherwise denies fevers/chills, night sweats, abdominal pain, nausea/vomiting or diarrhea.
He reports a history of childhood asthma but has not had symptoms since and is not on any inhalers. He is a homosexual male and has been monogamous over the past year and sexually inactive over the past 4 months. He gets regular testing for STDs, most recently 1 year ago. He routinely uses barrier contraception but ~6 months ago had an unprotected sexual encounter with his partner. He denies any IV drug use.
On physical exam, he is thin but not cachectic and in no acute distress. Hix exam is only notable for dry mucous membranes and small white plaques on his hard palate and in his posterior oropharynx.
His initial labs reveal a normal BMP. CBC shows leukopenia with WBC of 2.3 with decreased lymphocytes.
What is your differential?
This patient has dysphagia (sensation of food getting stuck), but more prominently, has odynophagia (pain with swallowing).
With the odynophagia, we were concerned this patient had esophagitis. Esophagitis can be infectious, erosive/reflux, inflammatory, medication/toxin related.
How would you manage this patient?
Given the presence of oropharyngeal candidiasis and concomitant odynophagia, we can empirically treat for esophageal candidiasis with oral or IV fluconazole. If he has no improvement after 72 hours of therapy, GI should be consulted for endoscopic evaluation to look for other causes of esophagitis.
What additional work-up would you want?
Presence of esophageal candidiasis in a patient who is not overtly immunosuppressed should prompt work-up for underlying immunocompromise including an HIV Ab/Ag screen and/or immunoglobulins.
His HIV Ab/Ag screen ultimately came back positive. His CD4 count was 4%.
- Esophageal candidiasis, in contrast to oropharyngeal candidiasis, does not occur in immunocompetent patients
- Presence of esophageal candidiasis should prompt further evaluation for:
- Presence of immunosuppressive medications (e.g., inhaled or systemic corticosteroids)
- Underlying immunodeficiency – HIV/AIDS, cancer, transplant
- In HIV patients, esophageal candidiasis is most commonly seen with CD4 < 200 and viral load > 400
- Thrush can be predictive of esophageal candidiasis2,4, but absence of thrush does not rule out esophageal involvement (particularly in HIV patients)3
- Most cases are caused by C. albicans
How is it diagnosed or treated?
- Improvement with empiric antifungal therapy
- Endoscopy should be done for patients who do not improve after 72 hrs of therapy.
- Endoscopy reveals thick white plaques and potential complications of esophagitis (strictures, ulcerations, esophagotracheal fistula)
Iti s treated with systemic antifungal therapy – fluconazole 400 mg loading dose, 200-400 mg daily for 14 – 21 days.
Quick note: Oropharyngeal candidiasis (thrush) can occur in patients who wear dentures, patients treated with antibiotics, prior head/neck radiation. It can also occur in patients on immunosuppressive agents (e.g., chemotherapy, steroids) or with immunodeficiency (e.g. HIV/AIDS).
A 44 yo M with a history of pemphigus vulgaris who presents with 4 days of severe pain with swallowing. He has a history of well controlled pemphigus vulgaris with cutaneous and oropharyngeal involvement. He was previously treated with 2 doses of rituximab ~ 8 months ago with good control of the disease. He has been on tapering steroids since his rituximab infusions and was decreased to 15 mg every other day ~ 1 month ago. He continues to have stable residual disease in two areas on his buccal mucosa and one small area on his scalp.
Four days ago, he noticed throat pain with swallow and an intense burning substernal pain. He also endorses a sensation of food being “stuck in his chest” that occurs with both liquids and solids. He reports thirst, feeling dehydrated, but no weight loss.
On physical exam, he has normal vital signs. His exam was notable for two small shallow ulcerations on buccal mucosa with a white base without surrounding erythema consistent with is residual pemphigus. No vesicular lesions were around oral mucosa or on lips. The rest of exam was unremarkable. His basic labs including BMP and CBC are all within normal limits.
What is your differential?
This is a patient with an underlying disease associated with mucosal involvement, on immunosuppression, who presents with symptoms concerning for esophagitis. A flare of pemphigus, erosive esophagitis (in the setting of steroid use), and infectious esophagitis are highest on the differential.
Differential of esophagitis includes:
GI was consulted and he underwent an EGD which showed several linear esophageal ulcerations.
Biopsies revealed IgG and C3 suprabasal intercellular deposition, suprabasal bullous formation consistent with pemphigus vulgaris. Fungal, CMV, and HSV testing were negative.
- Pemphigus vulgaris is rare autoimmune disorder that causes blistering
- Autoantibodies against desmoglein, an important protein in the epithelium causes flaccid blisters on the skin and mucosal surfaces
- Almost all patients have mucosal involvement
- Oropharynx is most commonly involved and is often presenting symptom
- Other mucosal surfaces involved include esophagus, vulva, vagina, anus, and conjunctiva
- Diagnosis relies on biopsy
TAKE HOME POINTS:
- Esophagitis can present as chest pain, odynophagia and dysphagia. Esophagitis can be reflux/erosive, infectious, inflammatory, or medication-related.
- Suspicion of esophageal candidiasis should prompt evaluation for underlying immunodeficiency (if not on systemic immunosuppression).
- Empiric oral fluconazole can be started on patients with a presumptive diagnosis of esophageal candidiasis. However, EGD should be done if no improvement of symptoms after 72 hours.
- Kauffman, CA. Clinical manifestations of oropharyngeal and esophageal candidiasis. In: Uptodate, Mitty, J (Ed), UpToDate, Waltham, MA. (Accessed on May 25, 2017).
- Pappas, PG, et al. 2009. Clinical Practice Guidelines for the Management of Candidiasis: 2009 Update by the Infectious Diseases Society of America. Clinical Infectious Diseases. 48:503-535.
- Nishimura, S, et al. 2013. Factors Associated with Esophageal Candidiasis and Its Endoscopic Severity in the Era of Antiretroviral Therapy. PLoS ONE, 8(3), e58217. http://doi.org/10.1371/journal.pone.0058217
- Samonis, G, et al. Oropharyngeal Candidiasis as a Marker for Esopahgeal Candidiasis in Patients with Cancer. Clinical Infectious Disease. 27 (2): 283-286.
- Kniesel, A & Hertl, M. 2011. Autoimmune bullous skin diseases. Part 1: Clinical manifestations. JDDG: Journal der Deutschen Dermatologischen Gesellschaft, 9: 844–857.