Annals of African Medicine, Vol. 2, No. 2, 2004, pp. 85-87

ADDISON’S DISEASE: A CASE REPORT

C. O. Alebiosu and O. Odusan

Department of Medicine, Olabisi Onabanjo University Teaching Hospital, Sagamu, Nigeria
Reprint requests to: Dr. C. O. Alebiosu, P. O. Box 4622, Dugbe Post Office, Ibadan, Oyo State, Nigeria E-mail: dralechristo@infoweb.abs.net ; dralechristo@yahoo.com 

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ABSTRACT

Addison’s disease is a rare endocrine disease. This is a report of a case of Addison’s disease as seen in a Nigerian female patient to highlight the occurrence in this environment as well as sensitize practitioners as to its possible aetiology, particularly in this era of tuberculosis and the pandemic of HIV/AIDS.  

Key words: Addison’s disease, tuberculosis

INTRODUCTION 

Addison’s disease is a rare endocrine disease¹ in which there is destruction of the adrenal cortex with resultant inadequate secretion of the adrenal cortical hormones-cortisol, aldosterone and androgens. Cortisol, the main hormone affected in the disorder is important in the body’s ability to cope with stressful situation such as infection, hypotension, and surgical procedures. Also involved and with an overriding influence on the adrenals is the hypothalamic–pituitary–adrenal axis. Addison’s disease is a term restricted to primary adrenocortical insufficiency. Other secondary or tertiary causes of adrenocortical insufficiency are not included in the term -‘Addison’s disease’. Primary adrenal insufficiency can be a life-threatening disorder particularly in stressful situation, since cortisol secretion cannot be increased on demand at all¹. The prevalence of primary adrenal insufficiency (Addison's disease) has been reported to be 39 to 60 per million population. ²

CASE REPORT

A 63-year-ol woman presented with increasing darkening of the skin, dizziness, and easy fatigability, nausea with occasional vomiting and progressive weight loss over eight months prior to presentation.  There were no headaches, blurred vision, and neither loss of consciousness nor change in her bowel habit. The medical history and systemic review revealed no abnormality and were not significant as to the likely cause of her disease state.

Physical examination revealed an elderly lady, pale, asthenic with generalized hyperpigmentation especially on the face, oral mucosa, palmar creases and knuckles. No features of malnutrition or hypovitaminosis. There was no significant peripheral lymphadenopathy. Main findings in the systemic examination were a pulse of 106 bpm, regular and small; blood pressure 100/60 mmHg supine and 70/40mmHg sitting. She could not stand on account of severe postural dizziness. The apex beat was normal.  Fundoscopy revealed a normal fundus. All other systems were essentially normal.  A clinical assessment of Addison’s disease to exclude paraneoplastic syndrome was made.

Laboratory investigations and results are shown in Table 1. Of note are the anaemia (haemoglobin-10gm/dl), with normal red cell morphology; ESR 58mm/hr (Westergreen method); fasting blood sugar was 76mg% and total serum protein of 7.8g/L (albumin-3.4g/L and globulin 4.4g/L). Plasma cortisol was undetectable at 0 and 30 minutes of cosyntropin administration (0.25 mg). Plasma rennin and aldosterone activity could not be estimated. HIV screening was negative (HIV 1 & II)

Radiological diagnostic tests included an abdominal ultrasound, which was reported as showing normal liver, spleen, pancreas and pelvic organs. However, the left kidney was not outlined. A computerised tomography scan (CT) of the abdomen showed a non-enhancing oval shaped left suprarenal mass with calcification and an ipsilateral hypoplastic but functional left kidney. There was neither ascites nor significant abdominal lymph nodes. The conclusion was a suprarenal tumour-adenoma or adrenocortical carcinoma to exclude tuberculous adrenalitis. A CT brain scan was normal.

She remained stable thereafter and made remarkable progress. After four weeks of admission, she was discharged to the medical outpatient clinic for further follow-up management. She had 3 episodes of Addisonian crisis after the initial hospital admission. These episodes were precipitated by acute malarial attacks, upper respiratory tract infection and gastroenteritis. Each episode, characterized by shock and altered consciousness, was managed with intravenous fluids (dextrose in saline), parenteral supplemental glucose, antibiotics, hydrocortisone and antimalarials where indicated. Each episode resulted in full recovery with no neurological deficit and was attended with rigorous counseling and health education of the patient and relatives as to the disease state and methods aimed at preventing this crisis. She has since remained in good health with lightening of the initial hyperpigmentation and also treatment of other clinical symptomatology. She is maintained on oral prednisolone, fludrocortisone and daily antituberculous medication.

DISCUSSION

Thomas Addison (1855)³ first described the clinical features of primary adrenal insufficiency, which may result from a variety of pathological processes.  Hence the term Addison’s disease. The characteristic form resulting from primary adreno-cortical insufficiency distinguishes Addison’s disease from other forms of adrenal insufficiency which may result from pituitary or hypothalamic diseases, with decrease in adrenocorticotrophic hormone (ACTH) secretion and consequent adrenal cortex atrophy.

The commonest causes of Addison’s disease are autoimmune and tuberculous adrenalitis. ¹ Others are fungal infection, metastatic neoplasia, haemochromatosis and congenital adrenal hyperplasia-adrenoleukodystrophy.  It is advised that tuberculosis should still be considered in the aetiology and diagnosis of adrenal insufficiency, particularly in areas where tuberculosis is endemic. ⁴ Between the mid-1980s and early 1990s, the synergistic combination of a deteriorating public health infrastructure worldwide, inadequate institutional control of infection, urban crowding and the epidemic of human immunodeficiency virus (HIV) infection resulted in a resurgence of tuberculosis, including infections with multidrug-resistant strains. ⁵ The development of active disease in persons with latent infection poses a continual threat of transmission, the control of which will be particularly difficult in regions where low case rates have resulted in decreased expertise in the identification and control of outbreaks of tuberculosis. ⁶ Currently, more than one third of the world's population is infected with M. tuberculosis; 8 million new cases and approximately 2 million deaths being reported each year. ⁷ Thus, it is expected that cases of extra-pulmonary manifestations of tuberculosis including the involvement of the endocrine system, should increase.

Addison’s disease may manifest with diverse andnon-specific clinical and/or biochemical features. ^{1, 9} The most specific sign of primary adrenal insufficiency is hyperpigmentation of the skin and mucosal surfaces associated with fatigue and weight loss. ¹ Soule reported that the presenting features among fifty patients seen over a 17-year period, as including hyperpigmentation (86%), weight loss (67%), abdominal pain (20%) and diarrhea (16%). ⁹ However, the disease may present atypically, requiring a high index of suspicion for diagnosis. ^1,10

The authors wish to express their sincere appreciation to Dr (Mrs.) Obajimi M, of the Radiological Department of the University College Hospital Ibadan, for reading the CT films.

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