1. Sjogren’s syndrome Iraj Salehi-Abari MD., Internist/Rheumatologist
Salehi I.

2. Sjogren’s syndrome: Definition:
A chronic autoimmune disorder with decreased exocrine gland function especially :
Diminished lacrimal gland function: Xerophthalmia
Diminished salivary gland function: Xerostomia
Salehi I.

3. Sjogren’s syndrome: Classified into: Previous name:
Primary Sjogren’s Syndrome
Secondary Sjogren’s Syndrome
The most common cause: RA
Previous name:
Mikulicz syndrome
Salehi I.

4. Clinical manifestations:
Exocrine gland features:
Sicca complex
Only feature: in 1/3 of cases
Extraglandular features
25%
Non-Hodgkin’s lymphoma
2.5%

5. Exocrine gland features:
Xerophthalmia
Xerostomia
Salivary gland enlargement
Vaginal dryness
dyspareunia
Xeroderma
Xerotrachea

6. Xerophthalmia(KCS): The most common cause of dry eyes as a disease
‘’gritty’’ or ‘’sandy’’ feeling in eyes
‘’dryness’’ of eye
Irritation, Itching
Photophobia
Mucus filaments at inner canthus
Corneal ulceration

7. Xerophthalmia(KCS): Infection of the eyelids
Punctate conjunctival & corneal damage
Rose Bengal staining
Fluorescein staining
Schirmer test
Dilation of the bulbar conjunctival vessels
Dullness of the conjunctiva & cornea

9. Rose Bengal Staining:

10. Xerostomia: Dry mouth Reduced salivary flow Dysphagia
Adherence of food to buccal surfaces
Changes in taste
Inability to eat dry food
Inability to speak continuously
Dental caries

11. Xerostomia: Gingivitis Oral candidiasis Angular cheilitis
Bacterial infections of stensen’s duct
Laryngotracheal reflux
Beefy red tongue
Chronic esophagitis

12. Salivary gland swelling:
It can be seen in 30-50% of cases
Glands are firm, diffuse and nontender
Chronic bilateral Parotid enlargement
Submandibular swelling
Sublingual swelling

13. Pathologic dry eyes:
The dryness of eyes is pathologic if there is at least one of below Features:
Duration of at least 3 months
Gritty or sandy sensation in the eyes
Use of a tear substitute more than 3 times daily

14. Etiology of Dry eyes:

15. Etiology of Dry eyes:

16. Pathologic dry mouth:
Dry mouth is pathologic if there is at least one of below Features:
Duration of at least 3 months
Patient has to wake up at night to drink water because his/her mouth is too dry
Patient frequently drinks liquids to help in swallowing dry foods

17. Dry mouth:

18. Salivary gland enlargement:
Unilateral:
Acute unilateral parotid swelling
Acute bacterial sialadenitis
Stones
Chronic unilateral parotid swelling
Tumors
Chronic bacterial sialadenitis
Actinomycosis

19. Salivary gland enlargement:
Acute bilateral parotid swelling:
Mumps
EBV
Echovirus
Coxsackie

20. Salivary gland enlargement:
Chronic (recurrent) bilateral parotid swelling:
Liver disease: Cirrhosis, Alcoholism
Infectious disease: TB, HCV and HIV infections
Nutritional disease: Malnutrition, Bulimia
Endocrine disease: Diabetes mellitus, Acromegaly
And
Rheumatology: Sjogren’s Syndrome, Sarcoidosis, Amyloidosis and IgG4-rsd

21. Bilateral parotid gland swelling:

22. Genitourinary features:
Vaginal dryness
Dyspareunia
Bacterial & candidal infections
Interstitial cystitis
Amenorrhea > 3 months
Menorrhagia/metrorrhagia
Endometriosis

23. Upper Airways: Recurrent nonallergic rhinitis & sinusitis Xerotrachea
Xerobronchia
Xerolaryngea

24. Extraglandular involvement:
Skin:
Xerosis (Xeroderma)
Palpable purpura
Raynaud’s phenomenon
Annular erythema(#SCLE)
Erythema Nodosum
Livedoreticularis
Lichen planus
Vitiligo
Cutaneous amyloidosis
Granuloma annulare
Angular cheilitis

25. Salehi I.

26. Joints: Muscles: Arthralgia + Arthritis : 50% Symmetric Nonerosive
Nondeforming
Intermittent
Hands & Knees
RF(+): 40%
Muscles:
Myopathy
Myositis

27. Thyroid disease: Autoimmune thyroid disease
Older, Female have higher incidence
No significant difference with NL population

28. Lungs: Heart: ILD: most common, asymptomatic LIP, NSIP
Acute pericarditis
LV hypokinesis
Heart block

29. Gastrointestinal tract:
Dysphagia
Esophageal dysmotility
Dyspepsia
Nausea
Epigastric pain
Atrophic gastritis
Achlorhydria & pernicious anemia
Celiac disease: 5%(10xNl)

30. Liver disease: PBC: Primary biliary cirrhosis Portal tract fibrosis
Chronic active hepatitis
Idiopathic portal hypertension

31. Kidney disease: Interstitial nephritis Renal tubular dysfuction:
RTA
Decreased concentrating ability
Rarely: Glomerulonephritis

32. Salehi I.

33. Bladder: Interstitial cystitis(20xNL) Dysuria Frequency Nocturia
urgency

34. Pancreatic disorder: Autoimmune sclerosing pancreatitis
Swelling of the pancreas
Narrowing of the pancreatic ducts

35. Peripheral Nervous System:
Polyneuropathy
Mononeuritis multiplex
Trigeminal neuropathy
Multiple cranial neuropathies
VII: Bell’s palsy
VIII: neural deafness
III, IV, VI

36. Central Nervous System:
Focal &/or Diffuse involvement
Spinal cord: Transverse myelitis
Subacute aseptic meningitis
Chorea

37. Chronic Fatigue Syndrome Fibromyalgia Syndrome Psychiatric disorder:
Depression
Personality disorders

38. Lymphoma: Non Hodgkin Lymphoma: 5%(16-44 x NL)
Diffuse Large B cell Lymphoma
Pseudolymphoma
Below features increase risk of lymphoma
Cutaneous vasculitis
Peripheral neuropathy
RF(+)
Type II cryoglobulinemia
Anti-Ro, Anti-La

39. Triad of: Cryoglobulinemia Hypocomplementemia Purpura
Correlates with severe disease complications & death

40. Pathogenesis: In a susceptible person:
Glandular epithelial cells express high levels of HLA-DR
This cells is APC
Antigens are Viruses or auto-Antigens
APCs  T-cell
CD4 T lymphocytes infiltrate in glandular tissue
Cytokine production
INFg & IL-2
T-cell  B-cell activation
Auto-Antibody production
Exocrine gland hypofunction
Increase B-cell malignancy

41. Salehi I.

42. Evaluations: CBC, BUN/Cr, Ast & Alt ESR, CRP, U/A
ANA, RF, Anti-Ro, Anti-La
Anti-RNP, Anti-Sm, Anti-dsDNA
C3 & C4, Cryoglobulins
Anti-alpha-fodrin Abs
Serum & urine Pr. Electrophoresis
Ig levels
HCV & HIV test
CXR, MRI of salivary gland enlargement
Schirmer’syndrome
Rose Bengal score
LGB

44. Diagnosis:
American-European Classification Group (AECG) classification criteria for Sjogren’s syndrome
American College of Rheumatology (ACR)-Sjogren’s International Collaborative Clinical Alliance (SICCA) classification criteria for Sjogren’s syndrome
IRAN criteria for early diagnosis of Sjogren’s syndrome: the best way

45. Diagnosis: Secondary Sjogren’s Syndrome:
A connective tissue disease plus
Ocular or Oral dryness and
At least 2 items out of below 3 items:
Ocular sign
Positive LGB (Biopsy)
Abnormal tests of salivary gland function

46. Treatment: Secondary SS: Primary SS: Severe disease:
Symptomatic therapy plus
Treatment of underlying disorder
Primary SS:
Disease modifying treatment
Severe disease:
Vasculits
Neuropathy
CTX + corticosteroids or Biologic agent