Autoimmune diseases is where the immune system of the body attacks its own healthy tissues, in this case the adrenal gland. Photo shutterstock.com

Dr Mattias Larsson*

Hằng, a vibrant and thriving 14-year old girl in the 8th grade, enjoys sports, has a great circle of friends and performs well in school.  However, she frequently feels stressed due to studies and loads of homework. The school nurse contacted Hằng's parents as she had gradually lost weight over the last few months and flagged a possible eating disorder. Her mother however thought that she ate well - in small portions, but frequently. Hằng had a craving for salty food and ate a lot of chips.

One morning Hằng woke up with high fever, fatigue, headache, cough and a sore throat. Her mother gave her some paracetamol and let her stay home. At breakfast, she felt nauseous and vomited. The paracetamol reduced the fever only slightly. As the fever continued the next day, her mother decided to take her to Family Medical Practice (FMP) for consultation. The paediatrician asked about the symptoms, investigated and ordered some tests. After 20 minutes they were called in and the doctor showed that she was positive for Influenza A. He prescribed oseltamivir, paracetamol and expectorant cough syrup. After three days of treatment the fever subsided and Hằng felt better.

However, after another two days she felt very tired again, had a severe stomach-ache, vomited several times and had diarrhea. Hằng also complained about lower back pain and felt dizzy. Her mother once again took her to FMP where they met the same paediatrician. During the symptoms inquiry, the doctor observed signs of fatigue in her expression, but she was able to respond adequately to questions. She had a high pulse and sore but soft abdomen, along with low blood pressure 80/45 mmHg. The skin exhibited low turgor (a delayed return to its normal position when pinched), and extended capillary refill (the time it takes for the blood to refill capillaries after being pushed away), both suggesting dehydration. The doctor also noticed areas of hyperpigmentation, darkened skin, especially over the elbows, knees, palms and knuckles.

Blood glucose tests showed low blood sugar, low sodium and high potassium. After asking some more questions about her condition and eating habits the doctor ordered some additional tests which showed low cortisol and aldosterone as well as high adrenocorticotropic hormone (ACTH) and renin. The doctor ordered intravenous fluids with the sugar dextrose.

The paediatrician explained that Hằng most likely had Addison's disease. This is an autoimmune disease where the body's own immune system attacks the adrenal gland, resulting in insufficient production of the steroid hormones cortisol and aldosterone by the adrenal glands.

The pediatrician also explained that Hằng may have an adrenal crisis with low blood pressure, vomiting, lower back pain, and effect on the consciousness. An adrenal crisis can be triggered by stress or an infection, like influenza.

The hyperpigmentation is caused by low cortisone levels causing a compensatory increase in the secretion of ACTH and MSH (melanocyte-stimulating hormone) from the adenohypophysis. The hormones increase the new synthesis of melanin. The hyperpigmentation is most visible on commonly exposed areas such as the knees and elbows.

The doctor further explained that treatment involves replacing the absent hormones with a synthetic corticosteroid, such as hydrocortisone or fludrocortisone. Her parents asked how long she would need to be treated. The doctor responded that the treatment is lifelong with continuous steroid replacement therapy with regular follow-up and monitoring for other health problems. A high-salt diet may also be useful for some people. With appropriate treatment, long-term outcomes are typically favourable, and most people are able to live a normal life. Without treatment, an adrenal crisis can result in death. Addison's disease affects about nine to 14 per 100,000 people and is more common in females. The disease is named after Thomas Addison, a graduate of the University of Edinburgh Medical School, who first described the condition in 1855.

Hằng was informed about her condition, at a follow up after three months she felt well, had gained weight and did not have abdominal pain. The parents were thankful that she got the diagnosis in time before any more complications occurred. Family Medical Practice 

Dr Mattias Larsson. Photo courtesy of Family Medical Practice

*Dr Mattias Larsson is a paediatric doctor at Family Medical Practice and associate professor at Karolinska Institutet and has a long experience in research on infectious diseases. He has worked with the Oxford University Clinical Research Unit and the Ministry of Health of Vietnam. He is fluent in English, Swedish, Vietnamese, German and some Spanish.

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