Connective Tissue DO's & Vasculitides Flashcards
What is one of the first tests that can be run to detect autoimmune disease?
Anti-nuclear Ab (ANA) by indirect immunofluorescence
Titer of <1.40 is normal
Female presents with malaise, malar rash, photosensitivity, and inflammatory arthritis. What is a possible diagnosis?
SLE
SLE common features
Fatigue Weight loss Malar Rash Inflammation Pericarditis (substernal crushing CP, worse when supine, ST elevation)
Patients with SLE are at an increased risk for what potentially fatal morbidity?
MI due to accelerated atherosclerosis
What labs would indicate SLE?
+ ANA
+ anti-ds DNA (follow)
+ Smith
decreased compliment levels due to consumption
SLE treatments
avoid sun exposure NSAIDs Glucocorticoids Hydroxychloroquine Minimize risks for atherosclerosis
What would be seen on an ophthalmic exam in patients with SLE/APS?
Cotton Wool Spots
Drug-Induced Lupus Mechanism
promotes demethylation of DNA
Drug-Induced Lupus labs
+ANA
+Anti-histone Ab’s (95%)
What are some medications that can induce Lupus?
Hydralazine Isoniazid Minocycline TNF Inh Quinidine Methyldopa Sulfa drugs (induce lupus but do not cause it)
A pregnant woman tests positive for anti-RO and anti-La. What is her newborn at risk for?
Neonatal Lupus
Describe Neonatal Lupus symtpoms
Transient: Rashes Thrombocytopenia Hemolytic Anemia Arthritis MONITOR FOR HEART BLOCK
What type of lupus is primarily seen on the head and face, and can develop into atrophic, disfiguring scars?
Discoid Lupus
What is the treatment for Discoid lupus?
Photoreception + anti-inflammatory agents or antimalarial drugs
A 50 year old F presents with thickening and hardening of the skin. Her skin is dry and itchy, and her fingers turn blue and white in response to cold. What is a possible diagnosis?
Scleroderma:
30-60 years
F>M
2 Raynauds Phenomenon
3 types of Scleroderma
- Localized
- Limited
- Diffuse
Which type of scleroderma is CREST syndrome seen in, and what does CREST stand for?
Limited Scleroderma (IcSSc) Calcinosis Raynauds (2) Esophageal dysmotility Sclerodactyly Telangiectasia
What is one of the primary risks associated with limited scleroderma?
Pulmonary Artery HTN
Renal Crisis uncommon
What is the a primary risk associated with diffuse system scleroderma?
Renal Crisis and Interstitial Lung Dz (velcro crackles)
What would you NOT prescribe for Diffuse Systemic Sclerosis?
High Dose glucocorticoids
*can induce renal crisis
Labs for Diffuse Scleroderma
+ ANA
+ Anti-Scl 70 (anti-DNA topoisomerase I)
+ Anti-RNA polymerase III
Labs for Limited Scleroderma
+ Anti-centromere
What should Sjogren’s patients avoid taking?
Atropinic drugs and decongestants
Dermatomyositis symptoms
Weakness w/o sensory loss Skin lesions: Gottrons patches Heliotrope rash (orbital edema) V-neck erythema (Shawl sign) Elevated CK & Aldolase
How does Dermatomyositis differ from Polymyositis?
NO skin changes seen with PM
Treatment for Inflammatory Myopathies
Glucocorticoids
Inclusion Body myositis
40-50 y/o
M>F
Weakness in finger flexion or quads
serology- anti-cN1A
How do we characterize systemic vasculitides?
Characterized by affected vessel size (small, variable, medium, large)
Small Vessel Vasculitides
IgAV (Hooch-Schonlein Purpura)
Good Pasture Syndrome
Granulomatosis with Polyangiitis (Wegener’s)
Variable Vasculitides
Eosinophilic Granulomatosis with Polyangiitis
Behcet Syndrome
Medium Vasculitides
Thromboangiitis Obliterates (Buerger Dz) Polyarteritis Nodosa Kawasaki Dz
Large vessel vasculitides
Takayasu Arteritis
Giant Cell Arteritis
What vasculitides is more common in children, and presents with the tetrad of 1) Purpura 2) Arthralgia 3) Abd pain, and 4) Renal Dz?
IgAV (HSP)
What disease is characterized by anti-basement membrane autoAb’s, diagnosed by UA, and can lead to pulmonary hemorrhage if not treated?
Goodpasture Syndrome
40 y/o M presents with granulomatous inflammation, glomerulonephritis, and epistaxis. Serology shows ANCA+, and vessels with granulomas. What is a possible Dx?
Wegener’s Granulomastosis (GPA)
Churg-Strauss Syndrome
Asthma + Eosinophilia
Purpura
Differentiate from Wegener’s by the elevated Eosinophil count
Behcet Syndrome
Triad: 1. recurrent mouth ulcers 2. genital ulcers 3. eye inflammation Serology: HLA-B51
Thromboangiitis Obliterates (Buerger)
Young males <35
ONLY OCCURS IN SMOKERS
Thrombosis and loss of digits
Polyarteritis Nodosa
Associated with HBV Peripheral Neuropathy with foot drop Renin-mediated HTN Livedo reticularis and digital gangrene Lungs are spared Serology: check for HBV
Kawasaki Dz
<5yo Japanese higher incidence
Aneurysm and MI
Presents with fever, inflamed “strawberry” tongue, lymphadenopathy, rash
tx: IVIG and ASA
What is referred to as Pulseless Disease?
Takayasu Arteritis:
<40yo
F>M
smooth tapered stenosis of large vessels
- renal artery stenosis
- retinopathy (copper-wiring)
- aortic complications
Giant Cell Arteritis
Large vessel (cranial Aa and Aortic arch)
>50yo
HA
Jaw Claudication
Increased ESR
START GLUCOCORTICOIDS IMMEDIATELY due to risk of blindness
temporal artery biopsy is gold standard for dx
Polymyalgia Rheumatica
Weakness secondary to pain
Elevated ESR and CRP
