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MSK 2 > Rheum d/o 2 > Flashcards

Rheum d/o 2 Flashcards

1
Q

Systemic chronic inflammatory disorder characterized by lymphocyte infiltration in EXOCRINE organs

A

Sjogren’s syndrome

MC present with SICCA symptoms and parotid gland enlargement

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2
Q

Sjogren’s syndrome extra glandular features

A

arthralgia, arthritis, myalgia and neuropathy

reynaud’s pehnomenon

lung, renal, GI dz

leukopenia, anemia, lymphadenopathy, vasculitis, lymphoma

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3
Q

cutaneous dz in Sjogren’s syndrome

A

50% of pts

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

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4
Q

SICCA symptoms

A

xerophthalmia, keratoconjunctivitis sicca, xerostomia

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5
Q

Sjogren’s syndrome associated diseases

A

SLE, RA

HCV, HIC

Thrush

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6
Q

epidemiology of Sjogren’s syndrome

A

MC women 40-60

insidious onset

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7
Q

PE of Sjogren’s syndrome

A

SICCA predominantly

diminished tear production, dull corneas w/dilated vessels

Buccal mucous and tongue are dry and fissured, increased THRUSH

bilateral parotid enlargement

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8
Q

arthralgia Sjogren’s syndrome

A

associated with non erosive arthritis

symmetric

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9
Q

thyroid disease in Sjogren’s syndrome

A

autoimmune thyroiditis MC

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10
Q

lung dz of Sjogren’s syndrome

A

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

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11
Q

GI issues Sjogren’s syndrome

A

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

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12
Q

systems affected by Sjogren’s syndrome

A 
Muscular 
Pulmonary 
GI
Renal
Thyroid
Neurologic 
Hematologic
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13
Q

malignancy and Sjogren’s syndrome

A

increased risk of NHL

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14
Q

labs in Sjogren’s syndrome

A
  1. elevated ESR, CRP
  2. ANA +
  3. Anti-Ro, Anti-La, SSB Abs
  4. RF _
  5. normocytic anemia
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15
Q

test done to diagnose SICCA/Sjogren’s syndrome

A

Schirmer test

less than 5 mm paper wet after 5 min

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16
Q

diagnosing Sjogren’s syndrome

A

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

17
Q

tx of Sjogren’s syndrome

A

artificial tears and water/candy to increase salvia

DMARDs used (if concomitant rheumatologic dz)

18
Q

GPA characterized by (3)

A
  1. systemic necrotizing angiitis of small and medium arterial vessels
  2. necrotizing granulomatous inflammation of respiratory tract
  3. necrotizing glomerulonephritis
19
Q

limited v widespread GPA

A

limited: just lungs
widespread: lungs + kidney

20
Q

GPA epi

A

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

21
Q

clinical presentation of GPA

A

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

22
Q

pulmonary GPA

A

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

can be asymptomatic, severe, fulminant

23
Q

pulmonary GPA s/s

A

pulmonary infiltrates
cough
hemoptysis
pleuritic chest pain and dyspnea

24
Q

systemic symptoms of GPA

A

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

25
Q

nervous system symptoms of GPA

A

sensory or motor deficits, CN palsies

vasculitis of small to medium sized vessels

26
Q

diagnostic criteria of GPA

A

2/4

nasal or oral inflammation

abnormal CXR findings

urinary sediment (microhematuria or RBC casts)

granulomatous inflammation on bx

27
Q

labs and GPA

A

non specific

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

28
Q

what serum testing done in GPA

A

P-ANCA and C-ANCA

most common C-ANCA positive

conferment with ELISA (r.o. good pastures)

29
Q

diffintintive test for GPA

A

tissue biopsy

30
Q

what does tissue biopsy show GPA

A

vasculitis or necrotizing granulomatous inflammation with little=no IG deposits in the granulomas

PAUCI IMMUNE NECROTIZING VASCULITIS

31
Q

MC cause of death in PGA

A

infection, respiratory and renal failure, malignancy and CV events

32
Q

tx of GPA (list) (5)

A
  1. steroids
  2. cyclophosphamide
  3. rituximab
  4. MTX
  5. plasmapheresis
33
Q

pharm tx GPA

A

steroid high dose for additive with rituximab

cyclophosphamide - only use is GPA, toxic side effects

MTX if mild and plasmapheresis if severe

34
Q

cyclophosphamide side effects

A

hemorrhagic cystitis
decreased fertility
bladder CA
other malignancy

35
Q

Rituximab and GPA

A

additive with steroids

monoclonal AB depleting B cells

36
Q

plasmapheresis and GPA

A

rapidly progressive renal dz

doesn’t improve survival/relapse rate but improves longtime survival/no dialysis

37
Q

remission and GPA

A

once induced, less toxic immunsuppresion and steroids used

but must give BACTRIM tablet to prophylaxis against PCP

38
Q

microscopic polyangitis

A

rare syndrome, confused with GPA

small-medium arteries and veins

P-ANCA + and increased durability of remission

39
Q

Churg Strauss

A

EGPA, small to medium sized arteries and veins

Asthma, eosinophilia and elevated IgE

p-ANCA positive

tx with steroids

MSK 2 (10 decks)

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