Sjogren's syndrome

Question 1

Sicca symptoms

Answer 1

associated with Sjogren’s syndrome of dry eyes and mouth

Question 2

what does Sjogren’s syndrome predispose a patient to getting?

Answer 2

increased risk for lymphoma (diffuse large B cell lymphoma, MALT (mucosa associated lymphoid tissue)

Question 3

if pt has Sjogren’s syndrome and new LAD, what do you get?

Answer 3

excisional lymph node biopsy to rule out lymphoma. need preserved architecture.

Question 4

exocrine features of Sjogren’s syndrome are:

Answer 4

keratoconjunctivitis sicca

dry mouth salivary hypertrophy

xerosis -dry skin

Question 5

keratoconjunctivitis sicca is

Answer 5

dryness of conjunctiva and cornea because too few tears are made

Question 6

extragrandular features of Sjogren’s syndrome?

Answer 6

Raynaud's phenomenon
cutaneous vasculitis
arthralgia/arthritis
interstitial lung dx
non hodgkin lymphoma

Question 7

diagnostic findings of sjogren’s syndrome?

Answer 7

objective signs of decreased lacrimation (schirmer’s test)
positive anti Ro (SSA) and or anti-La (SSB)
salivary gland biopsy with focal lymphocytic sialoadenitis

Question 8

primary vs secondary Sjogren’s syndrome?

Answer 8

classification:

Primary if not associated connective tissue disease

Secondary if comorbid connective tissue disease (SLE, RA and scleroderma)

Question 9

suspicious lab findings concerning for Sjogren’s dx

Answer 9

dry eyes and dry mouth with hoarseness, fatigue and elevated gamma globulin gap (total protein >4) think Sjogren’s syndrome

Question 10

most common renal manifestation of Sjogren’s syndrome is

Answer 10

chronic interstitial nephritis. - will have mild renal insufficiency and benign UA

See distal renal tubular acidosis (leading to metabolic acidosis with hypokalemia) and nephrogenic diabetes insipidus (low urine osmolality and frequent urination and excessive thirst)

Question 11

someone who presents with dry eyes and frequent urination and excessive thirst. Also see hoarse voice

Answer 11

think Sjogren’s with nephrogenic diabetes insipidus.

Question 12

what antibodies are associated with Sjogren’s syndrome?

Answer 12

positive anti Ro (SSA) and or anti-La (SSB)

Question 13

hypergammaglobulinemia is

Answer 13

seen in MM but also many collagen vascular disorders and autoimmune disorders due to chronic inflammation.

Question 14

chronic hepatitis C can cause

Answer 14

a SS like syndrome and renal manifestations but HCV will cause a nephrotic (large proteinuria or nephritic (hematuria or RBC casts) on UA. Sjogren’s will have a bland UA due to the chronic interstitial nephritis.

Question 15

xerophthalmia is

Answer 15

dry eyes

Question 16

xerostomia

Answer 16

dry mouth

Question 17

dry eyes, dry mouth, parotid gland enlargement and dentla caries

Answer 17

Sjogren’s syndrome

Question 18

longstanding Sjogren’s syndrome (>6 hrs) increases their overall risk for:

Answer 18

non hodgkin lymphoma

Question 19

lymphoma is from

Answer 19

malignant transformation of lymphoproliferative grandular tissue in SS

Question 20

risk factors for non hodgkin lymphoma in Sjogren’s syndrome:

Answer 20

positive anti-Ro/SSA anti/La SSB antibody positivity
RF positivity
cryoglobulinemia positivity
hypocomplementemia
lymphocytopenia

Question 21

primary NLH is seen with

Answer 21

Sjogren’s syndrome and presents in the parotid gland and is a low marginal zone B cell lymphoma

Question 22

diagnosis of lymphoma related to Sjogren’s syndrome is

Answer 22

excisional biopsy of the LN

Question 23

an impacted sialolith is seen with

Answer 23

pain, swelling and erythema

may also have fevers and chills with an infected salivary duct.

Question 24

pleomorphic adneomas are

Answer 24

common benign salivary gland tumors.

Question 25

Sjorgen’s dx seen with these labs:

Answer 25

RH +
ANA +
SSA, SSB+
elevated ESR

Question 26

what is Sjogren’s associated with

Answer 26

RTA 1 (distal)
see celiac edx

increased risk for lymphoma