Pocket Medicine: The Massachusetts General Hospital Handbook of Internal Medicine (110 page)

•
Dry eyes
(keratoconjunctivitis sicca): ↓ tear production; burning, scratchy sensation •
Dry mouth
(xerostomia): difficulty speaking/swallowing; dental caries; xerotrachea; thrush •
Parotid gland enlargement
: intermittent, painless, typically bilateral •
Vaginal dryness
and
dyspareunia

•
Recurrent nonallergic rhinitis/sinusitis
due to upper airway gland involvement •
Extraglandular manifestations
: arthritis; interstitial nephritis (40%); type I RTA (20%); cutaneous vasculitis (25%); neuropathies (10%); PNS or CNS disease; ILD; PBC

• ↑ risk of lymphoproliferative disorders (~50× ↑ risk of lymphoma and WM in 1° Sjögren’s)
Diagnostic studies

• Autoantibodies:
ANA (95%),
RF (75%)

Primary Sjögren’s:
anti-Ro
(anti-SS-A, 56%) and/or
anti-La
(anti-SS-B, 30%)

•
Schirmer test
: filter paper in palpebral fissures to assess tear production •
Rose-Bengal
staining: dye that reveals devitalized epithelium of cornea/conjunctiva •
Ocular staining score
: substitute for Rose-Bengal staining to determine degree of keratoconjunctivitis sicca using fluorescein and lissamine green •
Biopsy
(minor salivary, labial, lacrimal or parotid gland): lymphoplasmacytic infiltration
Classification criteria
(2 of 3 have 93% Se & 95% Sp;
Arthritis Care Res
2012;64:475)
1.
anti-Ro or anti-La or RF + ANA>1:320

2. Labial salivary gland bx w/ lymphocytic sialadenitis and score >1 focus/4 mm
²

3. Keratoconjunctivitis sicca w/ ocular staining score ≥3

Treatment

• Ocular: artificial tears, cyclosporine eyedrops

• Oral: sugar-free gum, lemon drops, saliva substitute, hydration, pilocarpine, cevimeline • Systemic: NSAIDs, steroids, DMARDs, rituximab

MIXED CONNECTIVE TISSUE DISEASE (MCTD)

Definition
(
Best Pract Res Clin Rheumatol
2012;26:61)
• Features of
SLE
,
systemic sclerosis
and/or
polymyositis
that appear gradually and often evolve to a dominant phenotype of SLE or systemic sclerosis • Different from undifferentiated CTD (UCTD): fail to meet criteria for any CTD; 30% go on to develop CTD over 3–5 y (usually SLE)
Clinical manifestations
(variable clinical course)
•
Raynaud’s phenomenon
typical presenting symptom (75–90%) • Hand edema (“puffy hands”), sclerodactyly, RA-like
arthritis
w/o erosions, polyarthralgias • Pulmonary involvement (85%) with
pulmonary hypertension
, fibrosis • Pericarditis most frequent cardiovascular manifestation; GI: dysmotility (70%) • Membranous & mesangial GN common (25%); low risk for renal HTN crisis or severe GN (if either, reconsider diagnosis of MCTD)
Diagnostic studies

•
ANA (>95%);
RF (50%);
anti-U1-RNP
in all, but not specific (seen in ~50% SLE)
Treatment

• As per specific rheumatic diseases detailed above

RAYNAUD’S PHENOMENON

Clinical manifestations
(
NEJM
2002;347:1001 & 2013;368:1344;
BMJ
2012;344:e289)
• Episodic, reversible digital ischemia, triggered by temp Δ (cold) or stress, classically:
blanching
(white, ischemia) →
cyanosis
(blue, hypoxia) →
rubor
(red, reperfusion); color Δ usually well demarcated; affects fingers, toes, ears, nose associated sx include cold, numbness, & paresthesias → throbbing & pain • Key to diagnosis and Rx is distinguishing between primary and secondary Raynaud’s
Primary
(80–90%=Raynaud’s disease; excluded all secondary causes)
• Onset 20–40 y,
>
(5:1); thought due to
functional
abnl of vessel wall • Clinical: mild, symmetric episodic attacks; no evidence of periph vascular disease;
no tissue injury
; nl nail-fold capillary exam; no systemic sx;
ANA; nl ESR