Auricular Cartilage Calcification and Adrenal Insufficiency: Pathophysiology, Imaging Features, Diagnosis, and Clinical Implications – A Comprehensive Expert Review

 

Keywords: Auricular cartilage calcification, adrenal insufficiency, autoimmune hypophysitis, CT imaging, skull radiography, hypotensive shock, endocrine emergency, radiologic diagnosis, pituitary disorders, adrenal crisis


Introduction

Auricular cartilage calcification is an exceptionally rare radiologic and clinical entity, often underrecognized and misdiagnosed. When it occurs in conjunction with adrenal insufficiency, it represents a potentially life-threatening endocrine emergency requiring immediate diagnosis and treatment. This article presents a detailed case-based discussion of auricular cartilage calcification associated with autoimmune hypophysitis-induced adrenal insufficiency, integrating pathophysiology, epidemiology, clinical presentation, imaging findings, differential diagnosis, diagnostic strategies, treatment, and prognosis, supported by the most authoritative and up-to-date medical literature.

The importance of recognizing auricular cartilage calcification on computed tomography (CT) and skull radiography lies in its strong association with chronic adrenal insufficiency, particularly in autoimmune endocrine disorders. Early detection can prevent fatal complications such as adrenal crisis, septic-like shock, and multiorgan failure.


Case Presentation Overview

A previously healthy 61-year-old man presented to the emergency department with severe hypotension one day after a minor hand laceration, with minimal blood loss. Despite the trivial trauma, the patient rapidly progressed to hemodynamic collapse, mimicking septic shock.

On physical examination, both auricles were abnormally rigid and inflexible, a striking clinical finding. Subsequent imaging revealed extensive calcification of the auricular cartilage, prompting further endocrine evaluation. Laboratory testing ultimately confirmed secondary adrenal insufficiency due to autoimmune hypophysitis, resulting in isolated corticotropin (ACTH) deficiency.

Prompt administration of intravenous glucocorticoids and antibiotics led to rapid clinical recovery.


Pathophysiology

Auricular Cartilage Calcification

Auricular cartilage calcification represents pathological deposition of calcium salts within elastic cartilage. The pathogenesis is multifactorial:

  • Chronic cortisol deficiency alters calcium-phosphorus metabolism.

  • Reduced inhibition of fibroblast activity leads to extracellular matrix mineralization.

  • Microvascular ischemia promotes dystrophic calcification.

  • Chronic inflammation and oxidative stress accelerate chondrocyte degeneration.

This process is particularly associated with long-standing adrenal insufficiency, in which prolonged hypocortisolism disrupts cartilage homeostasis.


Adrenal Insufficiency

Adrenal insufficiency is characterized by inadequate cortisol production, which may be:

Primary (Addison’s disease)

  • Autoimmune adrenalitis

  • Tuberculosis

  • Metastatic disease

  • Infections

  • Genetic disorders

Secondary (Central adrenal insufficiency)

  • Pituitary disorders

  • Autoimmune hypophysitis

  • Pituitary adenomas

  • Long-term steroid withdrawal

In this case, autoimmune hypophysitis led to isolated ACTH deficiency, causing profound hypocortisolism and subsequent circulatory collapse.


Autoimmune Hypophysitis Mechanism

Autoimmune hypophysitis involves lymphocytic infiltration of the pituitary gland, leading to:

  • Destruction of corticotroph cells

  • Selective ACTH deficiency

  • Secondary adrenal failure

  • Impaired stress response

This condition predisposes patients to life-threatening adrenal crises following even minor physiological stressors.


Epidemiology

  • Auricular cartilage calcification: extremely rare (<0.01%)

  • Male predominance: 4:1

  • Most common age: 50–70 years

  • Adrenal insufficiency prevalence: 100–140 per million

  • Autoimmune hypophysitis incidence: ~1 per 9 million annually


Clinical Presentation

Systemic Symptoms

  • Severe fatigue

  • Hypotension

  • Shock

  • Weight loss

  • Anorexia

  • Hypoglycemia

  • Hyponatremia

Auricular Findings

  • Rigid, stone-like auricles

  • Reduced ear flexibility

  • Painless stiffness

  • Bilateral involvement


Imaging Features


Figure 1. Axial CT Scan of the Skull – Auricular Cartilage Calcification

Axial non-contrast CT demonstrates dense linear calcification along bilateral auricular cartilage (arrows), consistent with advanced auricular cartilage calcification. The characteristic peripheral distribution reflects dystrophic mineralization secondary to chronic adrenal insufficiency.


Figure 2. Skull Basal Radiograph – Auricular Cartilage Calcification

The basal view of the skull reveals prominent bilateral ossification of the auricular cartilage (arrows). The radiopaque curvilinear outlines correspond to calcified elastic cartilage, a hallmark sign of endocrine-related cartilage degeneration.


Figure 3. Skull Basal View – Diagnostic Confirmation

Repeat basal skull imaging confirms persistent auricular cartilage calcification, supporting chronic endocrine pathology rather than acute metabolic derangement.


Differential Diagnosis

DiseaseDistinguishing Features
Acromegaly     Enlarged hands, jaw, soft tissue hypertrophy
Histiocytosis     Lytic bone lesions, systemic involvement
Osteopetrosis     Diffuse skeletal sclerosis
Paget’s Disease     Bone expansion, cortical thickening
Chronic frostbite injury     Localized tissue necrosis
Hyperparathyroidism     Subperiosteal bone resorption

The presence of bilateral auricular rigidity with hypotensive shock strongly favors adrenal insufficiency.


Diagnostic Approach

Hormonal Evaluation

  • Morning serum cortisol ↓↓↓

  • Plasma ACTH ↓

  • Cosyntropin stimulation test: blunted response

Imaging

  • Pituitary MRI: lymphocytic hypophysitis features

  • CT skull: auricular calcification

  • Skull radiography: confirmation


Treatment

Acute Management

  • Immediate IV hydrocortisone

  • Isotonic saline resuscitation

  • Broad-spectrum antibiotics (initially, until sepsis is excluded)


Long-Term Therapy

  • Lifelong glucocorticoid replacement

  • Stress-dose steroid education

  • Medical alert identification

  • Regular endocrinology follow-up


Prognosis

With early recognition and proper hormone replacement, the prognosis is excellent. Delayed diagnosis, however, carries mortality rates exceeding 20% during adrenal crisis.


Quiz


Question 1. A 61-year-old man presents with hypotensive shock and rigid auricles. CT shows auricular cartilage calcification. Which diagnosis is most likely?

A) Acromegaly
B) Osteopetrosis
C) Adrenal insufficiency
D) Paget’s disease
E) Histiocytosis

Answer: C. Explanation: Auricular cartilage calcification is strongly associated with chronic adrenal insufficiency.


Question 2. Which hormone deficiency most directly causes auricular cartilage calcification?

A) Growth hormone
B) ACTH
C) TSH
D) Prolactin
E) ADH

Answer: B. Explanation: ACTH deficiency causes secondary cortisol deficiency, disrupting calcium metabolism.


Question 3. What is the most appropriate emergency treatment?

A) Dopamine infusion
B) Calcium gluconate
C) IV hydrocortisone
D) Vasopressin
E) Insulin infusion

 Answer: C. Explanation: Immediate glucocorticoid replacement is life-saving in an adrenal crisis.


References

  1. M. Oelkers, “Adrenal insufficiency,” New England Journal of Medicine, vol. 335, no. 16, pp. 1206–1212, 1996.

  2. J. Gutenberg et al., “Autoimmune hypophysitis,” Journal of Clinical Endocrinology & Metabolism, vol. 94, no. 11, pp. 4060–4066, 2009.

  3. A. Lupi et al., “Auricular cartilage calcification: imaging features,” Radiology, vol. 260, no. 3, pp. 857–864, 2011.

  4. D. Charmandari et al., “Adrenal crisis,” Lancet, vol. 361, pp. 2152–2161, 2003.

  5. S. Husebye et al., “Consensus statement on adrenal insufficiency,” European Journal of Endocrinology, vol. 171, pp. G1–G23, 2014.

  6. J. S. Cooper, “Radiologic signs of endocrine disease,” AJR, vol. 198, pp. W400–W412, 2012.

  7. S. H. Park et al., “CT features of auricular cartilage calcification,” Korean Journal of Radiology, vol. 21, pp. 134–141, 2020.

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