Sjogren's / IgG4-RD Flashcards
SS pathogenesis models
- 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
SS infiltrating cells:
CD4+ T lymphocytes w/ memory B lymphocytes
(Others: plasma cells, CD8+, T reg, NK cells, DCs)
** Things that can be dry in Sjogren’s **
- Skin - xerosis, pruritus
- Eyes
- Mouth
- GI tract (constipation)
- Larynx (hoarse)
- Upper airway (nonallergic rhinitis, sinusitis, bleeding)
- Vagina: dyspaurenia
Ocular sx of primary SS
-Xeropthalmia or Keratoconjuntivitis sicca → infxn, ulceration, vision loss
-Painful/itchy eyes
-Sensation foreign body
-Blurred vision
-Photophobia
-Worse at night (vs blepharitis = mornings)
Tests for dry eyes
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)
Dry mouth sx in SS
-Taste: metallic, salty, bitter, burning sensation
-Difficulty swallowing
-Reflux
-Lipstick sign
-Infxn: caries, candidiasis
-Cracked teeth, loose fillings, trouble with dentures
Salivary gland tests in SS
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)
Biopsy requirements of MSG
4+ glands
-Minimum gland area 8mm2
Sjogren’s gland biopsy histopathology
Lymphocytic sialadenitis ( ≥1focus score ie ≥1 50 lymphocytes/4mm2)
-Acinar atrophy
-Ductal dilatation and fibrosis
-Epimyoepithelial cell islands
Meds causing dry eyes/mouth
Drugs with antimuscarinic activity:
- Antihistamines
-BZD
-TCAs
-drugs for overactive bladder, urinary incontinence
-Diuretics
Conditions causing dry eyes/mouth
-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
Glandular swelling DDX
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
UNILATERAL cause salivary gland enlargement
Infection (bacterial)
-Cancer: Lymphoma, primary salivary gland Ca
-Chronic sialadenitis
-Obstruction
ASYMMETRIC bilateral cause salivary gland enlargement
- Granuloma dz
-Infxn: HIV, mumps, CMV, HFlu, Coxackie A
-Recurrent parotitis of childhood
-IgG4
SYMMETRIC bilateral salivary gland enlargement
- Acromegaly
-Alcoholism,
-Chronic pancreatitis
-Hepatitis cirrhosis
-Anorexia/bulimia
-DM
-Gonadal hypofunction
-Idiopathic
** Exocrine glands involved in primary SS (and consequences)**
Skin (xerosis, pruritus)
-Upper airway (nonallergic rhinitis, sinusitis, bleeding)
-Larynx (hoarseness)
-Trachea (dry cough)
-GI (dysphagia, constipation)
-Vagina (dyspareunia)
Arthritis characteristics of primary SS
Similar to RA BUT nonerosive and mild
-Symmetric
-MCP, PIP
-AM stiffness
** Extraglandular manifestations primary SS **
- Constitutional, lymphadenopathy, lymphoma
- Neuro: CNS, peripheral neuropathy
- Autoimmune thyroid
- Lung
- GI: dysphagia, liver involvement
- Renal
- Vasculitis
- Raynaud
- MSK: arthralgia, arthritis, myositis
** Most common cryo types in SS**
Type 2 > 3 (mixed) poly IgM, poly IgG
Lung manifestations primary SS
-Xerotrachea/xerobronchitis
-ILD:NSIP, UIP, Lymphocytic interstitial pneumonitis
-Bronchiolitis (constrictive)
-Bronchiectasis
-Recurrent PNA
-Bronchial associ’d lymphoid tissue lymphoma
** Renal manifestations primary SS**
-Tubular interstitial nephritis (MC)
-Nephrogenic DI
-Type 1 RTA (hypoK, muscle paralysis, stones, high urine Ca and PO4),
-GN
** Renal Tx**
TIN: steroids alone, MMF
-GN: steroids, RTX, PLEX
GI manifestations primary SS
Celiac (think SS if diarrhea, vit D)
-PBC
-Autoimmune hepatitis
-Recurrent pancreatitis
Vasculitis of primary SS
MC: Cutaneous palpable pupura
-Infreq: necrotizing or cryo
Neuro manifestations of primary SS
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)
3 types neuropathy in Sjogens
- 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)
Neuropathy Tx
Pulse steroids + Ritux or Cyclo +/- IVIG
-Maintainence: MMF, AZA, Ritux