After diagnosis, patients with PGA-1 require close monitoring. Monitoring can help prevent illness associated with the delayed diagnosis of additional autoimmune diseases (eg, AD and hypoparathyroidism, which can develop during adulthood), as well as oral cancer (which may develop if candidiasis is not treated aggressively) and infection caused by asplenism, which is present in a subgroup of patients.
At present, the treatment of polyendocrine autoimmune syndromes is dictated by the treatment of the individual disorders and usually includes lifelong hormone replacement therapy for affected organs as well as close monitoring of the rest of the endocrine organs because they can also become affected. Knowledge of the syndromes allows early therapy of component disorders.
Thyroxine treatment for hypothyroidism increases the basal metabolic rate and therefore the body's requirement for cortisol; treating such cases of hypothyroidism can precipitate an episode of Addisonian crisis in a patient with undiagnosed adrenal insufficiency in the setting of PGA. CMC usually requires lifelong antifungal therapy, usually a topical one. It can also respond to systemic therapy, such as fluconazole or ketoconazole. Reports from several Hungarian studies have shown a good response to therapy with cyclosporin.[14,15,16]
The patient in this case was hospitalized and adequate hydration was given. A local antifungal was provided for the thrush, and iron was multivitamins were given. She was placed on oral steroids after initially receiving intravenous steroids. The patient and her parents were educated and counseled about the disease, its treatment, follow-up, and the side effects of treatment.
At her first follow-up visit 10 days after being discharged from the hospital, the patient had greatly improved both subjectively and objectively. The patient's vital signs were within normal limits, with a regular pulse of 86 beats/min and blood pressure of 110-120/70-80 mm Hg. She had marked improvement in her dizziness and no longer complained of abdominal pain or vomiting. Her thrush had disappeared, and she had gained 1.1- 2.2 lb (0.5 -1 kg) over the span of 10 days. Her hemoglobin level improved to 11.70 gm/dL.
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