Other Rheumatic Conditions Flashcards
Sjogren syndrome - overview
*a systemic autoimmune disease characterized by destruction of exocrine glands (especially lacrimal and salivary) by lymphocytic infiltrates
*divided into 2 categories:
-primary: without other connective tissue disease
-secondary: secondary to another autoimmune disease (RA, SLE, systemic sclerosis)
Sjogren syndrome - epidemiology
*predominantly affects females age 40-60 yo
Sjogren syndrome - pathogenesis
*combination of many things (environmental trigger, genetic component/susceptibility, etc)
*results in autoantibody production → stimulation of inflammation response → lymphocytic infiltration in organs:
-predominantly CD4+ T cells
-primarily TH1 driven
-type IV hypersensitivity reaction
Sjogren syndrome - clinical symptoms
- exocrine gland dysfunction: oral dryness, eye dryness (SICCA symptoms)
- extra-glandular dysfunction:
-MSK, pulmonary, cardiac, GI, renal, hematologic, endocrine
Sjogren syndrome - oral symptoms
*reduced salivary flow rate (xerostomia; oral sicca) → inability to speak continuously; gum disease; dental caries; halitosis; dysphagia
*mucosal atrophy
*salivary gland enlargement
*recurrent parotitis
Sjogren syndrome - eye symptoms
*history: using tear substitutes > 3x daily, “sand or gravel feel” in eyes
*note that dry eyes does not necessarily mean Sjogren’s
*Sjogren’s dry eye diagnosed by ophthalmologist: “keratoconjunctivitis sicca”
testing for keratoconjunctivitis sicca in Sjogren’s syndrome
- Schrimer test:
-objective measurement of tear production
-sterile filter paper placed on margin of eye
-measure wetting of paper after 5 min (<5mm is positive) - Rose Bengal dye:
-stains devitalized areas of the eye
-score based on what is involved
-potentially toxic so not used often
Sjogren’s syndrome - extraglandular involvement
*primary biliary cirrhosis
*Non-Hodgkin’s Lymphoma
*many others (MSK, cutaneous, pulmonary, cardiac, GI, renal, etc)
Sjogren’s syndrome & increased risk of lymphoma
*risk of lymphoma increased 44x in pts with Sjogren’s syndrome
*clues for malignant transformation:
-loss of previously elevated rheumatoid factor (RF)
-monoclonal gammopathy
*poor prognostic factors for development of lymphoma: parotid enlargement, vasculitis, hypocomplementemia, cryoglobulinemia
diagnosis of Sjogren’s syndrome
*multiple classification criteria, none very good at classifying the disease
*aspects involved in various criteria: subjective symptoms, objective clinical findings, abnormal serology, abnormal histology
Sjogren’s syndrome - labs
*ANA + (most pts)
*anti-Ro (SSA) and anti-La (SSB) can be seen
*can be RF+
*negative anti-CCP
*elevated ESR/CRP (most pts)
*can also see:
-hypergammaglobulinemia
-monoclonal gammopathy
-hypocomplementemia
Sjogren’s syndrome - minor salivary gland biopsy
*lymphocytic sialadenitis (focal lymphocytic infiltrates) with CD4+ T cells consistent with Sjogren’s syndrome
*seen on biopsy of lip
*there is a scoring system
Sjogren’s syndrome - treatment
- symptomatic treatment:
-beware of drug side effects
-artificial tears/nocturnal gels
-cyclosporine eye drops
-artificial saliva
-skin moisturizers - stimulate glandular secretion (pilocarpine, cevimeline)
- frequent follow up (dentist, ophthalmologist)
- other options
Sjogren’s syndrome - ddx
*infiltrative diseases (sarcoidosis, amyloidosis)
*IgG4-related disease
*graft vs. host disease
*diabetes mellitus
*hypertriglyceridemia
*chronic infection (HCV, HIV, HTLV-1)
*lymphoma: non-Hodgkin’s
*radiation therapy
sarcoidosis - epidemiology
*more common in African Americans (caucasians tend to be more asymptomatic)
*genetic component
sarcoidosis - clinical symptoms
*50% are found incidentally with CXR
*lungs most commonly involved site:
-cough, dyspnea on exertion, and chest pain
-hilar lymphadenopathy on CXR
*other systems involved:
-lymph nodes, skin rashes, arthritis, uveitis, etc
sarcoidosis - diagnosis
*ALWAYS biopsy of involved tissue (unless it is Lofgren syndrome): non-caseating granulomas (TH1 CD4+ helper T cells, IL-2, and IFN gamma)
*gallium scan to look for uptake (Panda sign)
*labs: no reliable labs; hypercalcemia, elevated ACE levels
Lofgren Syndrome - clinical presentation
*triad of symptoms:
1. erythema nodosum
2. hilar adenopathy
3. migratory polyarthralgia
*often young, predominantly female
note - this is the exception to the “always biopsy” rule of sarcoidosis
Lofgren Syndrome - diagnosis, prognosis, treatment
*95% diagnostic specificity for sarcoidosis when all 3 triad symptoms are present; no need to biopsy
*treatment: NSAIDs alone
*good prognosis, likely to go into remission
sarcoidosis - treatment
*steroids are mainstay
*may need steroid-sparing agents (methotrexate, azathioprine, TNF inhibitors)
*screen for eye disease and heart disease
antiphospholipid antibody syndrome - epidemiology
*70-80% women
*more common in Caucasians
*can occur as a primary disease or in association with SLE
antiphospholipid antibody syndrome - clinical presentations
- APS with vascular events (venous or arterial clots)
- APS with only pregnancy morbidity (recurrent miscarriages, pre-eclampsia, clotting of the cord)
- catastrophic APS (3 organ systems within a week)
note: asymptomatic aPL positivity, incidentally found, is NOT antiphospholipid antibody syndrome (need symptoms to have disease)
antiphospholipid antibody syndrome - Sapporo Criteria
- clinical criteria: vascular thrombosis OR pregnancy morbidity (1+ unexplained death of normal fetus > 10 wks; 1+ premature births due to pre-eclampsia, eclampsia, or placental insufficiency; 3+ unexplained consecutive spontaneous abortions)
- laboratory criteria (2 occasions, 12 weeks apart):
*anti-cardiolipin antibody
*lupus anticoagulant (DRVVT)
*anti-beta 2 glycoprotein antibody
antiphospholipid antibody syndrome - other manifestations
*Libman-Sacs Endocarditis: sterile platelet thrombi, mitral valve most commonly
*thrombocytopenia
*positive Coomb’s
*false positive VRDL
*seizures, TIA/CVA
*livedo reticularis
*clots in renal system
catastrophic antiphospholipid antibody syndrome
*thrombosis at multiple organ sites (3 or more) occurring concurrently or over one week
*these people are very very sick
*high mortality
antiphospholipid antibody syndrome - labs
*anti-cardiolipin antibody
*lupus anticoagulant (DRVVT)
*anti-beta 2 glycoprotein antibody
2 occasions, 12 weeks apart
lupus anticoagulant testing
- aPTT prolonged:
-patient’s serum is tested
-if not prolonged, test is negative - mixing study:
-mix with normal plasma
-if corrects, factor deficiency
-if prolonged, inhibitor present - add phospholipids:
-if corrects of improves, phospholipid dependent inhibitor
antiphospholipid antibody syndrome - treatment
*long-term anticoagulation with warfarin (poor efficacy seen with newer anticoagulants)
*catastrophic APS: steroids, heparin, plasmapheresis, immunosuppression, antibiotics
