II. Pathophysiology

  1. Idiopathic destructive Thyroiditis resulting in fibrosis of Thyroid and adjacent neck
  2. May be associated with more generalized fibrosis
    1. Idiopathic Multifocal Fibrosclerosis

III. Epidemiology

  1. Onset age 30 to 60 years

IV. Symptoms

  1. Symptoms are related to local compression on Esophagus and trachea (typically symptoms are more significant than the actual Goiter size)
  2. Compressive symptoms
    1. Stridor or Dyspnea
    2. Dysphagia
    3. Hoarseness

V. Signs

  1. Very firm (rock or wood-like) Thyroid swelling (Goiter)
  2. Hypothyroidism (30%)

VI. Labs

  1. Thyroid Function Tests (T4 Free and TSH)
    1. Most patients are euthyroid (70%)
    2. Hypothyroidism (Increased Serum TSH and decreased T4 Free) in up to 30% of cases
  2. Antithyroid peroxidase antibodies (>60%)
  3. Serum Calcium
    1. Hypocalcemia occurs if fibrosis affects the adjacent Parathyroid Glands

VII. Imaging

  1. Radioiodine Uptake (Thyroid uptake and scan)
    1. Low uptake (contrast with Grave's Disease)

VIII. Diagnosis

  1. Thyroid biopsy

IX. Management: Tracheal or esophageal compression

  1. First-line
    1. Surgical decompression
  2. Other agents (no definitive treatment)
    1. Corticosteroids
    2. Mycophenolate (Cellcept)
    3. Methotrexate
    4. Tamoxifen (suppresses fibroblast proliferation)

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