Other ANA+ Diseases Flashcards
What are the other ANA+ diseases
Sjogren’s
Systemic Sclerosis
Mixed Connective Tissue Disease
Undifferentiated connective tissue disease
Characteristics of ANA tests
Reported in a titer
Tested with other antibodies
very sensitive not very specific
High false positive rate
What medications can effect ANA test
IVIG, sulfasalazine, TNF-alpha inhibitors
What other things can lead to false ANA+ test
Infections, inflammatory liver diseases, cancer
Aging
Vit D deficiency
Tobacco smoke, silica, organic pollutants
dense fine speckled 70 (DSF70) associated with allergic disease
Other diseases associated with ANA+
Hashimoto's thyroiditis Type I diabetes Addison's Autoimmune anemia Psoriasis Lichen planus
What are Sjogren’s demographics/characteristics
Female»male (9:1)
Chief concern: dry eyes, dry mouth
Primary - no other underlying autoimmunity
Secondary - also have things like RA (15-20%)
What are the eye symptoms of Sjogren’s
Significant eye dryness - gritty sensation, burning eyes, worse thru the day
What are the mouth symptoms of Sjogren’s
Oral dryness - frequently drink water, increased dental carries, taste change, burning mouth, difficulty swalowing
Parotid/submandibular gland swelling
Other symptoms of Sjogren’s
Vaginal dryness Fatigue Arthralgias/arthritis Raynaud's phenomenon Dysphagia/esophageal dysfunction lymphadenopathy
less:
SOB, leg swelling, urinary changes, numbness/tingling/weakness, rash, B symptoms (fevers, night sweats, weight loss)
Other diseases that cause dryness
Diabetes mellitus dehydration blepharitis (eye inflammation) hepatitis C, HIV/AIDS depresssion/anxiety cystic fibrosis radiation to the head/neck
Medications that cause dryness
Antihistamines Tricyclic antidepressants benzodiazepines clonidine diuretics
Sjogren’s HEENT exam findings
Ocular injections, corneal damage, swelling of eye lids
Oral dryness, decreased salivary pooling, dental decay
Swelling in submandibular glands
Other Sjogren’s exam findings
Lymph: examine for adenopathy
MSK: arthritis
Skin: vasculitic lesions
Neuro: muscle strength and sensation
Sjogren’s labs
ANA+ (85-90%) SSA (50-70%), SSB (30-50%) Rheumatoid factor (sometimes) Elevateed Sed Rate Polyclonal hypergammaglobulinemia
What tests do you do to diagnose Sjogren’s
Schrimer's filter paper Fluorescein dye/lissamine green to eval conjunctiva Unstimulated salivary flow Ultrasound or MRI to eval parotid Minor salivary gland biopsy
Sjogren’s glandular symptoms treatment
Eye and dental care Humidifier, hydration, artificial saliva Eye drops, lubricants punctual plugs Meds: Pilocarpine, Cevimeline
Sjogren’s extra glandular treatment
Fatigue - rule out other medical cause
Arthritis - similar to RA: methotrexate, hydroxychloroquine, DMARD
Severe disease - rituximab, cyclophosphamide
Sjogren Pregnancy complications
SSA/SSB + = risk for neonatal lupus
Sjogrens complications for cancer
Increased 5-44 fold
median 8 years after diagnosis
Systemic Sclerosis demographics and characteristics
Female>Male
Age 35-64
High rates in Native American Tribes (esp. Choctaw)
Chief complaint: Skin thickening and tightening, severe Raynaud’s, fingertip ulcerations
Skin changes in SS
Limited or diffuse Tightening, thickening Distal arms, face, neck Early signs are puffiness or swelling Telangiectasias Raynaud's
Systemic Sclerosis Pulmonary signs
SOB, dry cough, leg swelling
Interstitial lung disease, more associated with generalized SCL 70 ab
Pulmonary hypertension - more associated with limited anticentromere ab
Systemic sclerosis GI symptoms
Severe GERD
Dysphagia
Decreased GI motility, small bowel bacterial overgrowth
GI bleeding/iron deficiency anemia (Gastric antral vascular ectasia aka watermelon stomach)
Systemic sclerosis MSK symptoms
Muscle weakness
Arthritis
Systemic sclerosis Renal symptoms
scleroderma renal crisis: RNA pol III
CREST syndrome
Calcinosis Raynaud's Esophageal dysmotility Sclerodactyly Telangiectasia
SS Physical exam
Vitals: hypertension
HEENT: tightening around face
Cardio: elevated JVP, loud S2, RV heave, TR murmur
Pulmonary: crackles/rales
Skin: thickening, sclerodactyly etc.
MSK: inflammatory arthritis/synovitis, acrolysis
SS Labs
ANA+ (85%)
ABs: ANA, anticentromere, Scl 70, RNA pol III
CXR, High res CT
Echocardiogram: pulmonary hypertension
Endoscopy or wallow evaluation for GI dysfunction
Tx SS Raynaud’s
Calcium channel blockers, topical nitroglycerin, sildenafil, pentoxifylline, fluoxetine
Tx SS GERD
PPI, H2 blockers
Tx SS Skin Dz
Methotrexate, cyclophosphamide, mycophenolate
Tx SS Lung dz
Mycophenolate, cyclophosphamide, antifibrotics, stem cell transplant
Tx SS Pulmonary hypertension
calcim channel blockers, diuretics, oxygen, prostacyclins, oral endothelin receptor antagonists, PDE inhibitors
Mixed Connective Tissue Disease demographics and characteristics
Femal > Male
Chief complaint: arthritis, Raynaud’s, puffy swollen hands, etc.
MCTD clinical symptoms
Arthritis SOB, chest pain, cough Rashes Muscle weakness Skin changes/severe Raynaud's 9rare to see Kidney disease)
MCTD Labs and Dx
ANA + (100%) High titer U1 RNP antibody (100%) Sometimes positive for RF, other ab CBC: may see anemia, leukopenia SPEP hypergammaglobulinemia Muscle symptoms = elevated creatinine kinase, aldolase rarely have low compliments
MCTD treatment
SLE-like: NSAIDS, Hydroxychloroquine, low dose steroids, methotrexate, azathioprine
SS-like: symptomatic Rx, ILD, mycophenolate, cyclophosphamide
Myositis: methotrexate, azathioprine, mycophenolate, steroids
PAH treatment (pulmonary vasodilators)
MCTD prognosis
58% stay diagnosed with MCTD, others evolve to SS, SLE, RA
Low mortality overall
Pulmonary hypertension is the highest risk
Undifferentiated Connective Tissue Disease Demographics and characteristics
25% of rheum patients do not meet criteria for difinable illness
Women 30-40 years old
Chief complaint: joint pain, others
UCTD most common symptoms
Joint pain
Raynaud’s
Rash/oral ulcers
SICCA symptoms
UCTD symptoms suggestive of other pathology
Fever, serositis, anti-dsDNA/Smith - SLE
abnormal nail capillaries, nuclear ANA - SS
Xerostomia, SSA/SSB - Sjogren’s
UCTD exam findings
Not enough to clearly suggest other pathology
UCTD Labs
ANA+
Other antibodies may be present (my indicate further progression)
UCTD treatment
Hydroxychloroquine
NSAIDs
low dose prednisone
Monitor - 25% progress to other pathology