Rheum d/o 2

Question 1

Systemic chronic inflammatory disorder characterized by lymphocyte infiltration in EXOCRINE organs

Answer 1

Sjogren’s syndrome

MC present with SICCA symptoms and parotid gland enlargement

Question 2

Sjogren’s syndrome extra glandular features

Answer 2

arthralgia, arthritis, myalgia and neuropathy

reynaud’s pehnomenon

lung, renal, GI dz

leukopenia, anemia, lymphadenopathy, vasculitis, lymphoma

Question 3

cutaneous dz in Sjogren’s syndrome

Answer 3

50% of pts

dry skin, purpora (2/2 vascultits and urticaria)

Question 4

SICCA symptoms

Answer 4

xerophthalmia, keratoconjunctivitis sicca, xerostomia

Question 5

Sjogren’s syndrome associated diseases

Answer 5

SLE, RA

HCV, HIC

Thrush

Question 6

epidemiology of Sjogren’s syndrome

Answer 6

MC women 40-60

insidious onset

Question 7

PE of Sjogren’s syndrome

Answer 7

SICCA predominantly

diminished tear production, dull corneas w/dilated vessels

Buccal mucous and tongue are dry and fissured, increased THRUSH

bilateral parotid enlargement

Question 8

arthralgia Sjogren’s syndrome

Answer 8

associated with non erosive arthritis

symmetric

Question 9

thyroid disease in Sjogren’s syndrome

Answer 9

autoimmune thyroiditis MC

Question 10

lung dz of Sjogren’s syndrome

Answer 10

interstitial lung dz, airway infection (lack of saliva) and persistent cough

Question 11

GI issues Sjogren’s syndrome

Answer 11

dysphagia (low saliva)
chronic atrophic gastritis
celiac
LFTs and PBC cholestatic pattern

Question 12

systems affected by Sjogren’s syndrome

Answer 12

Muscular 
Pulmonary 
GI
Renal
Thyroid
Neurologic 
Hematologic

Question 13

malignancy and Sjogren’s syndrome

Answer 13

increased risk of NHL

Question 14

labs in Sjogren’s syndrome

Answer 14

1. elevated ESR, CRP
2. ANA +
3. Anti-Ro, Anti-La, SSB Abs
4. RF _
5. normocytic anemia

Question 15

test done to diagnose SICCA/Sjogren’s syndrome

Answer 15

Schirmer test

less than 5 mm paper wet after 5 min

Question 16

diagnosing Sjogren’s syndrome

Answer 16

3+ months of dry eyes/mouth + combo of symptoms, labs, imaging and biopsy

Question 17

tx of Sjogren’s syndrome

Answer 17

artificial tears and water/candy to increase salvia

DMARDs used (if concomitant rheumatologic dz)

Question 18

GPA characterized by (3)

Answer 18

1. systemic necrotizing angiitis of small and medium arterial vessels
2. necrotizing granulomatous inflammation of respiratory tract
3. necrotizing glomerulonephritis

Question 19

limited v widespread GPA

Answer 19

limited: just lungs
widespread: lungs + kidney

Question 20

GPA epi

Answer 20

35-55, caucasians, Northern European, more common in men

Question 21

clinical presentation of GPA

Answer 21

appear ill and may be febrile with weight loss, fatigue, and night sweats

disease of eyes, sinus, pharynx, nasal mucosa is common (mimic infection) NO ORAL ULCERS

lung disease (mimic PNA) = hoarseness, stridor, wheezing, dyspnea

Question 22

pulmonary GPA

Answer 22

recurrent infection symptoms without culture (chronic sinusitis), fail to improve w/ tx

can be asymptomatic, severe, fulminant

Question 23

pulmonary GPA s/s

Answer 23

pulmonary infiltrates
cough
hemoptysis
pleuritic chest pain and dyspnea

Question 24

systemic symptoms of GPA

Answer 24

fevers, night sweats
fatigue, lethargy
loss of appetite, weight loss

Question 25

nervous system symptoms of GPA

Answer 25

sensory or motor deficits, CN palsies vasculitis of small to medium sized vessels

Question 26

diagnostic criteria of GPA

Answer 26

2/4 nasal or oral inflammation abnormal CXR findings urinary sediment (microhematuria or RBC casts) granulomatous inflammation on bx

Question 27

labs and GPA

Answer 27

non specific proteinuria, hematoria, elevated ESR, CRP, +ANA, RF+

Question 28

what serum testing done in GPA

Answer 28

P-ANCA and C-ANCA most common C-ANCA positive conferment with ELISA (r.o. good pastures)

Question 29

diffintintive test for GPA

Answer 29

tissue biopsy

Question 30

what does tissue biopsy show GPA

Answer 30

vasculitis or necrotizing granulomatous inflammation with little=no IG deposits in the granulomas PAUCI IMMUNE NECROTIZING VASCULITIS

Question 31

MC cause of death in PGA

Answer 31

infection, respiratory and renal failure, malignancy and CV events

Question 32

tx of GPA (list) (5)

Answer 32

1. steroids 2. cyclophosphamide 3. rituximab 4. MTX 5. plasmapheresis

Question 33

pharm tx GPA

Answer 33

steroid high dose for additive with rituximab cyclophosphamide - only use is GPA, toxic side effects MTX if mild and plasmapheresis if severe

Question 34

cyclophosphamide side effects

Answer 34

hemorrhagic cystitis decreased fertility bladder CA other malignancy

Question 35

Rituximab and GPA

Answer 35

additive with steroids monoclonal AB depleting B cells

Question 36

plasmapheresis and GPA

Answer 36

rapidly progressive renal dz | doesn't improve survival/relapse rate but improves longtime survival/no dialysis

Question 37

remission and GPA

Answer 37

once induced, less toxic immunsuppresion and steroids used but must give BACTRIM tablet to prophylaxis against PCP

Question 38

microscopic polyangitis

Answer 38

rare syndrome, confused with GPA small-medium arteries and veins P-ANCA + and increased durability of remission

Question 39

Churg Strauss

Answer 39

EGPA, small to medium sized arteries and veins Asthma, eosinophilia and elevated IgE p-ANCA positive tx with steroids