Srogren's Flashcards
What are some of the clinical characteristics of Sjorgren’s syndrome?
- Keratoconjunctivitis sicca (dry eyes)
- Xerostomia
- Parotid Gland Swelling
What are some characteristics to look for in someone that you suspect has primary Sjorgren’s?
Other than Dry eye, Dry mouth, and Parotid gland swelling you should look for:
• Raynaud’s
• Arthritis/Arthalgias
• Intersitial Lung Disease
• Neuropathy
• Purpura
AKA look for someone with dyspnea, cold and numb hands, painful joints with purple spots.
What is the characteristic histologic finding in Sjorgren’s?
• Mononuclear cellular infiltrate into the Lacrimal or salivary glands are the key histopathologic finding in these patients.
What are some very common causes of dry mouth and dry eye that may mimic the symptoms or Sjorgren’s?
• What do you need to actually diagnose someone with Sjorgren’s?
Anticholingerics (atropine, etc.) and Benedryl (1st gen. antihistamines)
• Because dry mouth, dry eye, and swollen parotid are ambiguous you need to do a biopsy of the lip and and an antibody screen for anti-SSA (anti-Ro), anti-SSB (anti-La) antibodies.
Who is most likely to present to you with Sjorgren’s syndrome?
• when you diagnose someone with Sjogren’s what should you also consider?
Most likely is a middle aged woman (9:1 F:M) that has dry eyes and dry mouth. There is a good chance she also has a concurrent autoimmune condition like SLE or even NON-HODGKIN’s LYMPHOMA (44x increased incidence).
Other than the typical symptoms of dry mouth and dry eyes, what are some other symptoms that people with Sjorgren’s might present with?
• Biliary tree inflammation/cirrhosis (pt. could be jaundice)
• Gastritis may be caused by a lack of mucous in stomach
• Skin and Vaginal dryness are also common
How do you expect the following systems to be affected in someone with Sjorgren’s syndrome?
• Renal
• GI
• Nervous System
• CV/Pulmonary
• Lymph
Renal Symptom:
• are rare, but may include RTA type I, GN, etc.
GI symptoms:
• esophageal dysfunction (Can’t eat a cracker), and biliary cirrhosis
Nervous System and Peripheral neves:
•may have problems with neuropathies, neuromyelitis optica is associated iwht anti-aquaporin-4 antibodies
CV/Pulmonary
• Vasculitis is rare
• Interstitial lung disease (fibrosis, pneuomonitis etc.)
Lymph:
• You really need to look out for MALT lymphomas
List the antibody marker that you’re likely to see in Sjorgren’s syndrome from most to least common.
- *75-95%** have positive RhF => non-specific
- *85%** have positive ANA => still pretty non-specific
- *33-50%** have anti-Ro (SSA) or anti-La (SSB) antibodies => much more specific
***Out side of this you may see low C4 and C3 complent from classical pathway activation and cryoglobulimenia or monoclonal gammopathies***
How does someone with keratoconjunctivitis sicca present?
• what tests would you do to confirm this is dry eye?
• These people often have extrememly dry, red, eyes that burn and are sensitive to light.
Diagnosis:
• BEST = Rose Bengal dye by an opthamologist that looks for epithelial lesions
• Schirmer’s test could also be used to monitor tear flow
Shown here are the ocular manifestations of Sjorgren’s.
How often do you expect to see Parotid and/or submandibular swelling in someone with Srogren’s?
Parotid and Salivary Gland Swelling is very common happening in about 50% of patients. This swelling is most often unilateral and episodic.
What are symptoms specific to the oral cavity that you might see in a patient with Sjrogren’s syndrome?
A very dry mouth with cavities and red ulcerated lips and tongue leading to difficulities chewing and swallowing.
What is shown here?
Snowflakes in the X ray of the parotid due to blockage from cellular infiltration
What are the potential complications of primary Sjogren’s?
Primary Sjogren’s may cause:
• Lymphocytic Infiltration into other organs to some extent
• Malignant transformation is possible