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1. Rheum RC > Sjogren's / IgG4-RD > Flashcards

Sjogren's / IgG4-RD Flashcards

1
Q

SS pathogenesis models

A
  • Lymphocytic infiltration of organs → cytotoxic cell death and apoptosis of acinar epithelium
  • ABs against muscarinic R type 3,
  • Cytokine breakdown of ACh
  • Altered aquaporin channel expression
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2
Q

SS infiltrating cells:

A

CD4+ T lymphocytes w/ memory B lymphocytes

(Others: plasma cells, CD8+, T reg, NK cells, DCs)

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3
Q

** Things that can be dry in Sjogren’s **

A
  • Skin - xerosis, pruritus
  • Eyes
  • Mouth
  • GI tract (constipation)
  • Larynx (hoarse)
  • Upper airway (nonallergic rhinitis, sinusitis, bleeding)
  • Vagina: dyspaurenia
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4
Q

Ocular sx of primary SS

A

-Xeropthalmia or Keratoconjuntivitis sicca → infxn, ulceration, vision loss
-Painful/itchy eyes
-Sensation foreign body
-Blurred vision
-Photophobia
-Worse at night (vs blepharitis = mornings)

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5
Q

Tests for dry eyes

A

Schirmer’s (<5mm in 5 min)
-Ocular surface staining (fluoresceine + lissamine green dye show cell disruption and damage; ≥5)
-Tear break up time (film BUT≤10s)

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6
Q

Dry mouth sx in SS

A

-Taste: metallic, salty, bitter, burning sensation
-Difficulty swallowing
-Reflux
-Lipstick sign
-Infxn: caries, candidiasis
-Cracked teeth, loose fillings, trouble with dentures

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7
Q

Salivary gland tests in SS

A

Sialometry (≤0.1cc/min)
-Scintigraphy (Tc-99m uptake/secretion)
-Sialography (anatomy; risk pain/infxn/duct rupture)
-MRI or US (parenchymal heterogeneity)
-Minor salivary gland biopsy (GOLD STD)

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8
Q

Biopsy requirements of MSG

A

4+ glands
-Minimum gland area 8mm2

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9
Q

Sjogren’s gland biopsy histopathology

A

Lymphocytic sialadenitis ( ≥1focus score ie ≥1 50 lymphocytes/4mm2)
-Acinar atrophy
-Ductal dilatation and fibrosis

-Epimyoepithelial cell islands

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10
Q

Meds causing dry eyes/mouth

A

Drugs with antimuscarinic activity:
- Antihistamines
-BZD
-TCAs
-drugs for overactive bladder, urinary incontinence

-Diuretics

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11
Q

Conditions causing dry eyes/mouth

A

-Amyloid, Sarcoid, IgG4RD
-Blepharitis
-Cystic fibrosis
-Dehydration
-Contact lens irritation
-Radiation
-DM
-GvHD
-Infxn: Hep C, HIV, TB, leprosy, mumps
-IgG4 disease
-Psychogenic: fear, depression
-Vit A def

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12
Q

Glandular swelling DDX

A

Autoimmune: sjogren’s, IgG4, granulomatous
-Infxn: bacterial, viral (influenza, HIV, CMV, mumps, coxsackie), TB,
-Ca: primary salivary gland Ca, lymphoma
-Endocrinopathy: acromegaly, DM, gonadal dysfcn
-Other: cirrhosis, pancreatitis, anorexia, bulemia, obstruction, recurrent parotitis, sialadenitis

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13
Q

UNILATERAL cause salivary gland enlargement

A

Infection (bacterial)
-Cancer: Lymphoma, primary salivary gland Ca
-Chronic sialadenitis
-Obstruction

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14
Q

ASYMMETRIC bilateral cause salivary gland enlargement

A
  • Granuloma dz
    -Infxn: HIV, mumps, CMV, HFlu, Coxackie A
    -Recurrent parotitis of childhood
    -IgG4
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15
Q

SYMMETRIC bilateral salivary gland enlargement

A
  • Acromegaly
    -Alcoholism,
    -Chronic pancreatitis
    -Hepatitis cirrhosis
    -Anorexia/bulimia
    -DM
    -Gonadal hypofunction
    -Idiopathic
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16
Q

** Exocrine glands involved in primary SS (and consequences)**

A

Skin (xerosis, pruritus)
-Upper airway (nonallergic rhinitis, sinusitis, bleeding)
-Larynx (hoarseness)
-Trachea (dry cough)
-GI (dysphagia, constipation)
-Vagina (dyspareunia)

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17
Q

Arthritis characteristics of primary SS

A

Similar to RA BUT nonerosive and mild
-Symmetric
-MCP, PIP
-AM stiffness

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18
Q

** Extraglandular manifestations primary SS **

A
  • Constitutional, lymphadenopathy, lymphoma
  • Neuro: CNS, peripheral neuropathy
  • Autoimmune thyroid
  • Lung
  • GI: dysphagia, liver involvement
  • Renal
  • Vasculitis
  • Raynaud
  • MSK: arthralgia, arthritis, myositis
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19
Q

** Most common cryo types in SS**

A

Type 2 > 3 (mixed) poly IgM, poly IgG

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20
Q

Lung manifestations primary SS

A

-Xerotrachea/xerobronchitis

-ILD:NSIP, UIP, Lymphocytic interstitial pneumonitis

-Bronchiolitis (constrictive)
-Bronchiectasis

-Recurrent PNA
-Bronchial associ’d lymphoid tissue lymphoma

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21
Q

** Renal manifestations primary SS**

A

-Tubular interstitial nephritis (MC)
-Nephrogenic DI
-Type 1 RTA (hypoK, muscle paralysis, stones, high urine Ca and PO4),
-GN

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22
Q

** Renal Tx**

A

TIN: steroids alone, MMF
-GN: steroids, RTX, PLEX

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23
Q

GI manifestations primary SS

A

Celiac (think SS if diarrhea, vit D)
-PBC
-Autoimmune hepatitis
-Recurrent pancreatitis

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24
Q

Vasculitis of primary SS

A

MC: Cutaneous palpable pupura
-Infreq: necrotizing or cryo

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25
Q

Neuro manifestations of primary SS

A

CNS: sclerosis-like brain lesion, sz, encephalopathy, cog impairment, sensory/motor deficit, longitudinal extensive transverse myelitis, optic neuritis

PNS: motor (mononeuritis multiplex), sensory, or sensorimotor
- Small fiber neuropathy (burning pain w/ normal NCS) → dysautonomia (loss pinprick, temperature, w/ normal vibration/DTR)

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26
Q

3 types neuropathy in Sjogens

A
  • Mononeuropathy multiplex
  • Polyneuropathy (axonal sensorimotor, chronic inflamm demyelinating polyneuorpathy eg CIDP; distal sensory)
  • Polyradiculopathy
  • Sensorimotor polyradiculoneuropathy ie. Guillain-Barré syndrome
  • Sensory (small fiber, sensory ataxic)
  • Cranial neuropathy (eg Bell’s)
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27
Q

Neuropathy Tx

A

Pulse steroids + Ritux or Cyclo +/- IVIG
-Maintainence: MMF, AZA, Ritux

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28
Q

MC SS neuropathy

A

Axonal sensorimotor polyneuropathy

29
Q

Tx of longitudinally extensive transverse myelitis

A

Induction: Pulsed steroids + ritux/CYC
-Maintenance: MMF, AZA, RItux

30
Q

Small fiber neuropathy Tx in SS

A

-Neuropathic meds: gabapentin, pregabalin, topical lidocaine, capsaicin

-Refractory: IVIG

31
Q

SS risks in pregannacy

A

Neonatal / Congenital heart block if Ro/La positive

32
Q

Lab findings in primary SS

A

ANA, Ro, La, RF, (some hv ACA- high risk extraglandular, Raynaud, lymphoma)
-Hypergammaglobulinemia
-AoCD, Leukopenia, Thrombocytopenia

33
Q

Cancer risks in SS

A
  • NHL
  • Mucosa-associated lymphoid tissue lymphoma (MALT)
34
Q

** SS clinical risk factors for lymphoma**

A

-Disease duration > 10y
-Moderate/high dz activity (EULAR DAI >5)
-Persistent salivary gland enlargement
-Lymphadenopathy
-Raynaud’s
-Splenomegaly
-Palpable purpura
-GN
-Peripheral neuropathy

35
Q

** SS lab risk factors for lymphoma **

A

-Monoclonal gammopathy (IgM kappa)
-Loss of previously positive RF
-Leukopenia
-Cryoglobulinemia
-Low C3/C4
-Presence of germinal centers or high focus score ≥3 on MSG biopsy

36
Q

Bloodwork for cancer screening SS

A

CBC, SPEP, RF, complements, cryo q1-2y

37
Q

** ACR/EULAR classification primary SS**

A

Positive if ≥ 4:
-Ocular staining score ≥ 5 (1)
-Schirmer <5mm/5min (1)
Unstim whole saliva flow rate ≤ 0.1/min (1)
Ro positive (3)
Labial salivary biopsy: focal score >1 (3)

38
Q

** ACR/EULAR 2016 classification inclusion criteria**

A

One symptom of ocular or oral dryness:
– Daily, persistent, troublesome dry eyes or mouth for >3mo OR
– Recurrent sand/gravel sensation OR
– Use tear substitute >3x/d OR
– Drink liquids to aid in swallowing food

39
Q

** ACR/EULAR 2016 classification exclusion criteria**

A

HEENT radiation
-Active Hep C or AIDS
-Sarcoid or Amyloid
-IgG4RD
-GvHd

40
Q

Tx xeropthalmia

A

Environment:
– Reduce caffeine, smoking, computer time, celing fans, offending meds
– Increase fluids, omega 3, humidifier
– Special eyewear
-
-Eye drops: preservative free artificial tears, topical steroid, cyclosporine, autologous tears
-Lubricant ointment: at night
-Punctal occlusion: silicone collagen (delay clearance)
-Scleral contact lens

41
Q

Tx blepharitis

A

Warm compress
-Avoid local irritant
-Topical Azithromycin
-Rare: systemic antibiotics (doxy)

42
Q

Tx dry mouth SS

A

Regular dental visit
-Hydration
-Avoid caffeine, EtOH, nicotine, acidic beverages
-Sugar free / Xylitol gum, lozenge, candy
-Remove nasal polyps (limit mouth breathing)
-Biotene mouthwash
-Saliva substitute
-Secretagogues

43
Q

Secretagogue examples & dose

A

Pilocarpine 5mg QID
-Cevimeline 30mg TID (muscarinic ACh R Ag)

44
Q

Secretagogue side effects

A

Flushing
-Diaphoresis
-GI disturbances (nausea, abdo pain, anorexia, constipation, GERD)
-Pinpoint pupils (ie dont give to narrow angle glaucoma)
-Rhinitis/sinusitis (ie dont give to asthma pt)
-Bradycardia (ie avoid BBs)
-Urinary frequency

45
Q

Who to avoid secretagogues in

A

Narrow angle glaucoma
-Asthma
-Pt on beta blockers

46
Q

Retroperitoneal fibrosis H&P, Ix

A

H: pain (lower back, abdo, flank, scrotum), fevers, anorexia, malaise

-P: HTN, LE edema, phlebitis

-Ix: ESR/CRP, azotemia, CT (mass around abdo aorta btwn renal arteries and aortic bifurcation, hydronephrosis, ureter deviation), Biopsy - sclerosis and mononuclear cell infiltration

47
Q

Retroperitoneal fibrosis 2ndary causes

A

Drugs: ergot, methysergide
-Malignant disease: lymphoma/sarcoma
-Erdheim Chester
-Infxn
-IgG4RD

48
Q

Retroperitoneal fibrosis Tx

A

High dose pred (taper to 10mg/d by 3-6mo), maintenance x1-3y
-Resistant: MTX, AZA, MMF, Tamoxifen

-Stent - relieves ureteral obstruction

-Surgery/Endovascular aneurysm repair for large aneurysm

49
Q

IgG4 HEENT involvement

A
  • Constitutional: fatigue, weight loss, arthralgias, enthesopathy (no synovitis/enthesitis)
  • CNS: pachymeningitis (dura inflammation), hypophysitis (pituitary involvement)

-Orbital pseudotumor, Lacrimal gland inflammation, Sicca, EoM myositis, Proptosis

-Salivary (sialadenitis, enlarged, sicca, SUBMANDIBULAR not parotid as in SS)

-Mikulicz dz - bilat painless swelling lacrimal and maj salivary glands

-ENT: allergy/atopy = rhinosinusitis, polyps, obstruction, asthma, eosinophelia, IgE

-Thyroiditis (fibrosis can infiltrate airways), hypothyroid

  • LN: nontender, generalized or localized. cervical, paraaortic, inguinal, retroperitoneal, supraclavicular etc. **DONT biopsy as low yield for fibrosis
50
Q

** IgG4-RD Lung involvement **

A
  • MC: Mediastinal and hilar lymphadenopathy
    -Central airway disease
    -Pleuritis, Pleural nodules, effusion, pleural thickening
    -Fibrosing mediastinitis
    -ILD (NSIP, GGO)
    -Inflammatory pseudotumor
    -Mediastinal/hilar adenopathy
51
Q

** IgG4-RD GI manifestations**

A

-MC: Type 1 autoimmune pancreatitis (sausage pancreas) → DM

-Sclerosing cholangitis → painless obstructive jaundice

52
Q

** IgG4-RD Renal manifestations**

A

MC: Tubulointerstitial Nephritis
Membranous GN
Prostatitis (BPH resolves after steroids)
Hydronephrosis from ureteral obstruction (from retroperitoneal fibrosis)

53
Q

IgG4-RD cardiac manifestations

A

-Pericarditis
-Periaortitis (especially infrarenal)
- Aorta (thoracic/abdo) aneurysm. Aortitis does not affect other branches (unlike LVV)
-Involvement of infrarenal aorta and branches (esp iliac arteries) eg stenosis

54
Q

** IgG4-RD Biopsy findings**

A
  1. STORIFORM fibrosis pattern
  2. Obliterative phlebitis(medium sized veins)
  3. CD4+ lymphocyte and IgG4+ plasma cell infiltration (lymphoplasmacytic infiltrate) - seen in cancer, AAV, castleman’s
  4. IgG4+ plasma cells > 40% of total IgG+ plasma cells. >10 IgG4+ plasma cells/hpf
  5. Tissue eosinophils

-**NO necrotizing vasculitis and granulomatous inflammation

55
Q

IgG4 dx

A

Must use clinical, labs, radiology and biopsy, none in isolation enough!

Biopsy + IgG4 serum [ ] >135mg/dL confirms

56
Q

IgG4 labs

A

Polyclonal gammopathy IgG and IgE
-IgG4 and mild eosinophilia

-Hypocomplementemia (bc IgG1 forms immune complex; present in renal dz)
-ESR high,
- CRP normal unless aortitis or retroperitoneal fibrosis)

RF, ANA can be positive

57
Q

**IgG4 exclusion criteria - clinical **

A

-Fever
-No response to steroids (40mg/d x4wks)

58
Q

IgG4 exclusion criteria - serologic

A

Leukopenia and thrombocytopenia w/o explanation (bc unusual in IgG4RD)
-Peripheral eosinophilia
-Cryo
-Positive antibodies! Eg PR3/MPO, Ro/La, dsDNA, RNP, Sm, Scl70, Jo1 (excludes RF, ANA, AMA, ASMA, APLA)

59
Q

IgG4 exclusion criteria - radiologic

A

Rapid progression (w/i 4-6wks)
-Findings suspicious for infxn,cancer, Erdheim chester (multifocal osteosclerotic lesions)
-Splenomegaly >14cm w/o explanation

60
Q

IgG4 exclusion criteria - pathologic

A

Cancer findings (atypia, light chain restriction)
-Findings of inflammation (granulomatous, neutrophilic, inflammatory myofibrobastic tumor)
-Features of macrophage/histiocytic d/o
-Necrosis, fibrinoid necrosis

61
Q

IgG4 exclusion criteria - diagnoses

A

Multicentric Castleman’s
-IBD (if only pancreatobiliary dz present)
-Hashimoto (if only thyroid affected)

62
Q

IgG4 Tx

A
  • Steroids (0.5mg/kg for induction , taper over 3-6mo; no less than 5-7.5mg for maintenance)
  • csDMARDs (MMF, LFN, AZA) still relapse
  • Ritux (1g 2 weeks apart or 375mg/m2 x 4 weekly infusion, then 1g q6mo)

DC after 3 years of clinical, radiological, lab remission

-Surgical/Endovascular aneurysm repair

63
Q

** Why Sjogren’s has polyclonal IgG**

A

Lymphocyte hyperactivitiy
Ag processed by APC activates T cells to induce proliferation of Ag-specific B cells
IL6 cause differentiation into AB-producing plasma cells that orliferate in BM, gut, LN, spleen, liver

64
Q

** HIV pt gets Sjogren’s – what is it and how is it different/similar from idiopathic SS?**

A

Diffuse Infiltrative Lymphocytosis Syndrome (DILS)
- Predominantly CD8+ T cells (vs CD4+ in SS) in blood and visceral infiltration
- Autoantibodies: Ro/La uncommon
- More common to have extraglandular features

Similar
- B/L parotid/lacrimal gland swelling
- Sicca

65
Q

What diseases can peripheral ulcerative keratitis happen in?

A

ie corneal melt

RA
Sjogren
Relapsing polynchondritis
GPA
PAN

66
Q

Differences between Sjogren’s and IgG4RD

A

Sjogren’s:
- Age 40-50s (vs 60s)
- F>M (vs M>F)
- Glandular swelling recurrent (vs persistent)
- Higher rate arthralgia and lymphoma

  • ANA+ (vs rare)
  • Ro/La+ (vs rare)
  • Normal levels IgG4 (vs high)
  • Normal IgE levels (vs high)
  • Mainly CD4+ T lymphocyte infiltration (vs IgG4+ plasmocytic infiltration)
  • Bx: fibrosis without storiform pattern, no obliterative phlebitis, no IgG4+ plastmacytes
67
Q

IgG4 Phenotypes from ACR/EULAR classification criteria

A
  1. Pancreatohepatobiliary: T1 autoimmune pancreatitis and sclerosing cholangitis
  2. Periaortitis, aortitis, retroperitoneal fibrosis
  3. HEENT limited - young asian females, orbital pseudotumors, ENT, thyroiditis, pachymeningitis, hypophysitis
  4. Mikulicz syndrome - Dacryoadenitis, sialednitis, ILD, pancreatitis, renal manifestations
68
Q

IgG4 Imaging

A

Gross organomegaly
Sausage pancreas
Infrarenal periaortitis
MRCP/ERCP showing irregular narrowing of bile ducts
PET to find sites of bx

69
Q

Igg4 predictors of relapse

A

high baseline Igg4 level
high baseline ige level
peripheral eosinophilia
mulitorgan involvement
young woman with head/neck limited dz

1. Rheum RC (23 decks)

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