Sjogren's syndrome
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Clinic
- SjS or SS, also known as Sicca is a long-term autoimmune disease that affects the body's moisture-producing (lacrimal and salivary) glands.
- Sjogren syndrome is characterized by disruption of epithelial cells and lymphoplasmocytic infiltration of exocrine glands
- Cutaneous manifestations include Vasculitis, Angular cheilitis, Eyelid dermatitis, Annular erythemam, Xerosis and Anhidrosis.
- Patients with SBIs have a diverse presentation of symptoms that may mimic SS, including dry eyes, dry mouth, and fatigue along with cutaneous complaints and pruritus. [1]
- It often seriously affects other organ systems, such as the lungs, kidneys, and nervous system.
- Affected patients may also experience complex neurological symptoms characterized by extremity paresthesia and pain as well as dizziness and presyncope.
- SjS may present initially with pure neurological symptoms due to experiencing severe autonomic neuropathy symptoms. [2]
Pathology
SS could have two different underlying mechanisms [3]
- Exocrinopathy: Autoimmune inflammation of salivary glands
- Autonomic nervous system dysfunction: Due to Autoantibodies against the muscarinic acetylcholine receptors
Sign / Symptoms
- Primary symptoms
- Dry mouth
- Keratoconjunctivitis sicca: Blurred vision, constant eye discomfort
- Pain
- Fatigue
- Other symptoms can include
- Dry skin
- Vaginal, Nasal dryness
- Chronic cough
- Numbness in arms and legs
- Feeling tired, muscle and joint pains
- Thyroid problems
- 15% increased risk of lymphoma
- Recurrent mouth infections, swollen parotid glands
- Dysphonia
- Dysphagia
- Autoimmune tubulointerstitial nephritis leading to proteinuria, urinary concentrating defect, and distal renal tubular acidosis.
- Hypokalemic paralysis due to primary Sjögren syndrome was rarely reported
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 | CMV | |
|---|---|---|---|---|---|---|
| Sialadenitis | + | +++ | +++ | ++ | ||
| Parotiditis | +++ | +++ | +++ | +++ | ||
| Hypotention/ Orthostatic | +++ | +++ | ||||
| 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.
- Here the only dynamic entity is Sialadenitis / Parotiditis. Others such as Keratitis, Conjunctivitis, Dysphonia, Dysphagia, Chronic Cough, Dry mouth/ Vagina are static consequence of mucosal dryness
- ↑ Gadarowski MB, Pukhalskaya T, Farah R, Smoller BR. Acquired anhidrosis in a patient with Sjogren syndrome and silicone breast implants. JAAD Case Rep. 2020 Apr 29;6(5):414-416. doi: 10.1016/j.jdcr.2020.02.037. PMID: 32382632; PMCID: PMC7200200.
- ↑ Chaaban N, Shaver T, Kshatriya S. Sjogren Syndrome-Associated Autonomic Neuropathy. Cureus. 2022 Jun 1;14(6):e25563. doi: 10.7759/cureus.25563. PMID: 35784971; PMCID: PMC9248235.
- ↑ Nikolov NP, Illei GG. Pathogenesis of Sjögren's syndrome. Curr Opin Rheumatol. 2009 Sep;21(5):465-70. doi: 10.1097/BOR.0b013e32832eba21. PMID: 19568172; PMCID: PMC2766246.