Sjogren's syndrome

Clinic

• SjS or SS, also known as Sicca
• It is a long-term autoimmune disease that affects the body's moisture-producing (lacrimal and salivary) glands
• It often seriously affects other organ systems, such as the lungs, kidneys, and nervous system.

Pathology

Interestingly SS could have two different underlying mechanisms

1. Exocrinopathy: Autoimmune inflammation of salivary glans
2. Autonomic nervous system dysfunction: Due to Autoantibodies against the muscarinic acetylcholine receptors

Skin dryness is the result of lymphocytic infiltration into skin glands

Sign / Symptoms

• Primary symptoms are :r
  • Hallmark symptom: Dry mouth and keratoconjunctivitis sicca (dry eyes).
  • Pain
  • Fatigue.
• Other symptoms can include
  • Dry skin, Vaginal, Nasal dryness
  • Chronic cough
  • Numbness in the arms and legs
  • Feeling tired, muscle and joint pains
  • Thyroid problems.
• 15% increased risk of lymphoma.
• Blurred vision, constant eye discomfort
• Recurrent mouth infections, swollen parotid glands, Dysphonia (vocal disorders including hoarseness), and difficulty in swallowing and eating.
• Autoimmune tubulointerstitial nephritis leading to proteinuria, urinary concentrating defect, and distal renal tubular acidosis.
• Hypokalemic paralysis due to primary Sjögren syndrome was rarely reported in the literature.

Related disease

• RA
• SLE
• Systemic sclerosis

• Celiac disease

• Fibromyalgia
• Autoimmune thyroiditis
• MS
• Spondyloarthropathy
• Several malignancies, principally non-Hodgkin lymphoma
• Type I cryoglobulinemia is a known complication of Sjögren's syndrome.

Entities / Miasms

	EBV	HCV	MMP	MTB	HTLV-1
Sialadenitis Parotiditis	+	+++	+++	++
Thyroiditis	+	+++	+++	+
Nephritis	++	++	++
Lymphoma	+++				+++

HTLV-1 is a good candidate for SS with autonomic neuropathy mechanism, while EBV / HCV / MMP / MTB are good glandular autoimmunity choices.