Sjögren's syndrome

Classic symptom triad

The pathology has a clearly defined clinical picture. The diagnosis is based on the presence of the classic triad of signs:

1. Xerostomia — a subjective feeling of severe dry mouth resulting from hypofunction of the salivary glands.
2. Dry keratoconjunctivitis (combined with xerophthalmia — dry eyes) — the result of decreased tear production.
3. Presence of a systemic autoimmune disease — the pathology does not occur in isolation, but accompanies such diseases as rheumatoid arthritis, systemic lupus erythematosus (SLE), or scleroderma.

Differences between Sjögren's syndrome and Sjögren's disease

In pathological anatomy and rheumatology, it is important to strictly differentiate between two related concepts of different nature.

• Sjögren's syndrome is always secondary. It is a concomitant symptom complex developing against the background of a pre-existing systemic autoimmune pathology.
• Sjögren's disease is a primary, absolutely isolated lesion exclusively of the salivary glands. The most important diagnostic marker of Sjögren's disease is the detection of specific antibodies directed against the epithelium of the salivary gland ducts in the patient's blood.

Pathogenesis and systemic involvement

The development of the disease is based on an autoimmune impairment of tear and saliva secretion. However, the pathological process is not always limited to the glandular tissue of the head and neck.

Since the syndrome is closely associated with systemic connective tissue diseases, other organs may also be involved in the pathological process. Possible systemic manifestations include:

• Joint involvement.
• Respiratory system involvement (lung damage).
• Gastrointestinal pathology (pancreatic involvement).

Sjögren's syndrome itself often acts as an extra-articular manifestation of other rheumatological diseases.

Association with other systemic manifestations

Sjögren's syndrome must be considered in the context of a wide range of extra-articular manifestations of autoimmune pathologies. When assessing clinical signs of systemicity (Red Flags), the physician pays attention not only to dry keratoconjunctivitis.

Simultaneously, the patient may present with rheumatoid skin nodules, cutaneous vasculitis (up to ulceronecrotic vasculitis and nail bed infarctions), polyneuropathies, pericarditis, or pleuritis. The presence of sicca syndrome requires the exclusion of such formidable pathologies as systemic lupus erythematosus (which is also characterized by uveitis, recurrent mucosal ulcers, renal involvement, and arthritis) or rheumatoid arthritis.

Differential diagnosis of hypofunction

When examining the salivary glands, Sjögren's syndrome is considered in the category of functional disorders, specifically — hypofunction.

For comparison, other pathologies of the salivary glands have a completely different pathogenesis and morphology:

• Obstructive lesions (sialolithiasis, mucoceles, retention cysts) cause secretion stasis and salivary colic. With a prolonged course of sialolithiasis, the function of the gland also decreases and ceases, but this is associated with parenchymal atrophy, connective tissue proliferation, and squamous/oncocytic metaplasia of the duct epithelium.
• Tumor-like lesions (sialadenosis, oncocytic change, Mikulicz's disease) and infections (mumps) lead to enlargement of the glands rather than a systemic "dry" syndrome.

Class and exam questions

What else to study on this topic

• Salivary gland stone disease (sialolithiasis) and mechanisms of stone formation
• Obstructive lesions: mucocele, ranula, and retention cysts
• Tumor-like lesions of the salivary glands (Mikulicz's disease, sialadenosis)
• Clinical signs of systemicity in autoimmune diseases (Red Flags)
• Extra-articular manifestations of rheumatological diseases

Other topics in the "Additional Topics" section

Prepared by WhiteCoat Pro faculty in accordance with the medical school curriculum. For educational purposes only.