Sjogren's syndrome: Difference between revisions

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=== Clinic ===
=== Clinic ===


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


=== Pathology ===
=== Pathology ===
Interestingly SS could have two different underlying mechanisms <ref>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.</ref>
SS could have two different underlying mechanisms <ref>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.</ref>


# Exocrinopathy: Autoimmune inflammation of salivary glans
# Exocrinopathy: Autoimmune inflammation of salivary glands
# Autonomic nervous system dysfunction: Due to Autoantibodies against the muscarinic acetylcholine receptors
# [[Autonomic Nervous System|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 ===
=== Sign / Symptoms ===
* Primary symptoms are :r
* Primary symptoms
**Hallmark symptom: Dry mouth and keratoconjunctivitis sicca (dry eyes).
**Dry mouth  
**Keratoconjunctivitis sicca: Blurred vision, constant eye discomfort
**Pain  
**Pain  
**Fatigue.
**Fatigue
* Other symptoms can include  
* Other symptoms can include  
**Dry skin, Vaginal, Nasal dryness
**Dry skin
**Vaginal, Nasal dryness
**Chronic cough
**Chronic cough
**Numbness in the arms and legs
**Numbness in arms and legs
**Feeling tired, muscle and joint pains
**Feeling tired, muscle and joint pains
**Thyroid problems.
**Thyroid problems
* 15% increased risk of lymphoma.
* 15% increased risk of lymphoma
* Blurred vision, constant eye discomfort
* Recurrent mouth infections, swollen parotid glands
* Recurrent mouth infections, swollen parotid glands, Dysphonia (vocal disorders including hoarseness), and difficulty in swallowing and eating.
* Dysphonia  
* Dysphagia
* Autoimmune tubulointerstitial nephritis leading to proteinuria, urinary concentrating defect, and distal renal tubular acidosis.
* 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.
* Hypokalemic paralysis due to primary Sjögren syndrome was rarely reported




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!MTB
!MTB
![[HTLV-1, Human T lymphotropic virus type 1|HTLV-1]]
![[HTLV-1, Human T lymphotropic virus type 1|HTLV-1]]
!CMV
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|''[[Sialadenitis]]''
|''[[Sialadenitis]]''
Parotiditis
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|[[Parotiditis]]
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|Hypotention/ Orthostatic
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|Thyroiditis
|Thyroiditis
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[[HTLV-1, Human T lymphotropic virus type 1|HTLV-1]] is a good candidate for SS with [[Autonomic Neuropathy entities|autonomic neuropathy]] mechanism, while EBV / HCV / MMP / MTB are good glandular autoimmunity choices.
 
* [[HTLV-1, Human T lymphotropic virus type 1|HTLV-1]] is a good candidate for SS with [[Autonomic Neuropathy entities|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

Latest revision as of 04:00, 4 May 2023

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]

  1. Exocrinopathy: Autoimmune inflammation of salivary glands
  2. 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
  1. ↑ 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.
  2. ↑ 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.
  3. ↑ 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.
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