Block 5 Case 1

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Plaquenil: full name, class, indications, MOA, adverse effects.: Hydroxychoroquine Sulfate
Antiprotozoals, antirheumatic
Malaria, Rheumatoid arthritis, SLE
Inhibits migration of neutrophils, eosinophils.
Retinopathy.
Fluconazole: class, MOA, adverse effects: Antifungal
Alters fungal membranes, inhibiting ergosterol synthesis.
Well tolerated.
Prednisone: class, MOA, adverse effects: Corticosteroid: antiinflammatory, immunosuppresent.
Binds to cytosolic receptors, effects gene transcription: reduced arachidonic acid, proinflammatory cytokines etc.
Hypothalmic-pituitary-adrenal axis suppression.
Orthostatic Hypotension: definition: 20mm Hg fall in systolic
10mm Hg fall in diastolic
Symptoms of cerebral hypoperfusion
Normal pulse pressure: 50 mm Hg
Widened pulse pressure: value and conditions: > 65mm Hg
Aortic regurg, hyperthyroid, anxiety, bradycardia
Narrowed pulse pressure: value and conditions: < 30 mm Hg
Tachycardia, severe aortic stenosis, constrictive pericarditis, paricardial tamponade
Schirmer test: Filter paper in lateral third of lower eyelid, measure fluid on paper after 5min. <5mm abnormally low.
Sjogren Syndrome: epidemiology: 2% adult population
9:1 female:male
60% secondary to other autoimmune: SLE, RA etc.
Sjogren Syndrome: pathophysiology: Chronic activation of immune system.. B Lympocytes hyperreactive.
Focal lymphocytic infiltrates seen around glandular ducts, consisting of CD4+ T cells, B cells and others. Infiltrate eventually extends to acinar epithelium, causing gland dysfunction.
Sjogren Syndrome: primary symptoms: Fatigue
Dry skin
Arthralgias
Neurologic involvement
44x Higher risk of lymphoma
Elevated ESR
Sjogren Syndrome: diagnostic criteria: 4 of 6:
Symptoms of dry eye
Abnormal Schirmer test
Symptoms of dry mouth
Tests of salivary gland function
Minor salivary gland biopsy
Autoantibodies (SS-A, SS-B)
Sjogren Syndrome: treatment: Muscarinic agonists to active M3 receptor -> secretion
Tear/saliva substitutes
Goggles to hold in eye moisture.
Antibody panel: RNP: SLE (30-40%) and mixed connective tissue
Antibody panel: SM: "Smith"
Specific to SLE in 10-40%
Antibody panel: SSA/Ro: Sjogren's (75%)
SLE (50%)
Antibody panel: SSB/La: Sjogren's (40-50%)
SLE (10-15%)
Congenital complete heart block (90%)
Neonatal Lupus (70%)
Antibody panel: SCL-70: Scleroderma (20-40%)
Antibody panel: ds-DNA: SLE (60-80%)
97% specific to SLE.
Causes of Nephrogenic DI: Genetic: V2 receptor defect, aquaporin-2 defect
Autoimmune disease: SLE, Sjogrens
Causes of Central DI: Brian tumor
Head Trauma
Granulomatous disease
Idiopathic (50%)
Vasopressin Challenge: dose: .05-.1 mL, intranasally
1 mcg IV
Synthetic ADHs: types, pharmacokinetics: Pitressin: acts on V1 receptor in gut
Desmopressin: acts on V2 receptor in kidney... like vasopressin but w/o vasopressor activity.

Must be given IV, nasally, inactivated by pepsin.
Threshold of dilute urine in DI... and sodium concentrations due to water loss...: < 250 mOsm/kg

> 142 mEq/L
Water restriction test: elevate serum osmalarity > 295 mOsm/kg while measuruing urine osmolarity. Then administer ADH and watch urine osmolarity.
"ADH dependent" DI drug therapy: Thiazide diuretics
NSAIDS (inhibit prostaglandin synthesis, promotes ADH action)
Mechanism of ESR: In inflammation, increased fibrinogen leading to RBC stacking... causing them to descend faster.
Antibodies seen in SLE: ANA
ds-DNA
Smith (Sm)
Antibodies seen in RA: Rh factor
high ESR
20% ANA
Antibodies seen in SS: SS-A (Ro)
SS-B (La)
Rh factor
Antibodies seen in systemic sclerosis: ANA
anti-Scl 70
Antibodies seen in mixed connective tissue disease: ANA
Anti-RNP
Antibodies seen in inflammatory myopathies (polymyositis, dermatomyositis): ANA
anti-Jo 1
RH factor
ESR
Antibodies seen in polyarteritis Nodosa: high ESR
ANA
ANCA (antineutrophil cytoplasmic antibody)
ADH Synthesis: Produced and packaged in ADH neurons in supraoptic nuclei of hypothalamus.

Stored in posterior pituitary.
ADH regulation: Osmoreceptors in anterior hypothalamus.
Baroreceptors in AA, LA, CA detect hypovolemia.
ADH actions: Increases water permeability of principle cells of late distal tubule by binding to G-ptn linked V2 receptor, inserting AQP2 in luminal membrane.

Also causes vascular smooth muscle contraction via V1 receptor G-ptn. Increases TPR.
Water deprivation test procedure: All water witheld
Urine osmolality and body weight measured hourly
Vasopressing given when 2 sequential urine Osm vary by <30mOsm.
Final urine obtained 60 min later for osmolality measurement.
Diagnostic criteria for SLE:: 4 of 11: SOAP BOX MD
Serositis, Oral ulcers, Arthritis, Photosensitivity

Blood disorders, Renal invovelemtn, Antinuclear antibodies, Immunologic phenominon, Neurologic disorders

Malar rash, Discoid rash
Histologic changes in Sjogren's syndrome:: lymphocytic infiltration, acinar loss... general preservation of ducts.
Sarcoidosis: epidemiology: ~20:100,000
Favors non-smokers
2:1 female:male
3x incidence 10x prevalance in african americans
Sarcoidosis: Pathogenesis: CD4+ T lymphs accumulate. They release IL-2 activating more. Nearby cells release TNF, IL-12 recruiting additional cells. Macrophages/epitheliod cells and MNG cells coalesce into granulomas.

Dysfunction caused by granuloma load, not release of inflammatory mediatiors into normal tissue.
Sarcoidosis: Clinical Manifestations: Systemic complaints
Erythema nodosum
Restrictive PFT pattern, diffusion problem.
Many others... all organs involved.
Sarcoidosis: Lofgren's syndrome: erythema nodosum, bilateral hilar adenopathy on CXR, joint symptoms
Sarcoidosis: Heerfort-Waldenstrom syndrome: fever, parotid enlargement, anterior uveitis, facial nerve palsy
Meaning of positive ANA and negative ANA: + indicates the need for further testing.
- usually indicates NO SLE or related autoimmune disease, especially at low titers.
Sarcoidosis: Select Diagnosis: ACE levels elevated (40-80%)
Mediastinal adenopathy on CXR
Skin anergy
Histologic demonstration of NCGs.
Sarcoidosis: Treatment: 1st line: Glucocorticoids
2nd line: Methotrexate/cyclosporine

Waiting... 50% cases last 12-36 months, most last <5 years.

10-15% last longer.
Differential for NCG: Sarcoidosis
Crohns
Fungal: histoplasmosis, coccidioidomycosis
Bacterial: Leprosy, Cat scratch, tertiary syphilis
Foreign body reaction
Morphology of NCGs: focal accumulation of activated macrophages, surrounded by lymphocytes and plasma cells.

Can be immune or foreign body based.
Bone marrow: normal WBC:RBC ratio: 3:1
Bone marrow: adult red marrow locations: axial skeleton & girdles

ribs, sternum, clavicles, vertebrae, pelvis, skull.
Bone marrow: functions: Hematopoiesis

Macrophages also clean blood
Lymph node: anatomical buzz words: germinal centers
capsule
afferent and efferent lymph vessels
sinuses
medullary chords
hilum
Lymph node: contents of germinal center: predominantly B lymphocytes. Surrounded by a mantle of T lymphocytes.
DEXA scan: measurements: BMD for lumbar spine, lateral lumbar spine, hip, total body, body composition, and spinal morphometry.
DEXA scan: length, dose: short, low dose
DEXA scan: terminology (BMD, BMC, Zscore, Tscore): Bone mineral density (normalized)
Bone mineral content (absolute)
Z is variation between age/sex matched
T is variation between young normal individuals
WHO osteoporosis classifications (normal, osteopenia, osteoporosis, severe osteoporosis): Normal: BMD/BMC < 1 SD below young adult mean
Osteopenia: 1 - 2.5 SD below
Osteoporosis: > 2.5 SD below
Severe Osteoporosis: > 2.5 SD below + 1 or more fragility fractures
Pathogenesis of GIOP (glucocorticoid induced osteoporosis): Decreased bone turnover.

GCs suppress production of osteoblasts, increase apoptosis of osteocytes and osteoblasts.

GCs inhibit intestinal Ca absorption and promote renal Ca excretion.

GCs suppress growth factors, which stimulate bone formation.
Prevention of GIOP.: Calcium + vitamin D
Baseline BMD with DXA scan
Start bisphosphonate therapy.
GIOP: bisphosphonate therapy: antiresorptive agents that decrease osteoclast activity and stimulate osteoclast apoptosis!
PFT volunes: VC, TLC, RV, FRV, ERV, IC: VC: max expiration after max inspiration
TLC: total lung capacity
RV: air left in lung after max expiration
FRV: amount left in lung after norm expiration
ERV: max air exhaled after normal expiration
IC: max air inspired after normal expiration
Flow volume loops: normal, obstructive, restrictive: norm: quick spike, linear drop, saddle recovery
obstructive: quick low spike, scouped out drop
restrictive: smaller loop (witch's hat)
PFT characteristics of obstructive: FVC < 80%
FEV1 low.
FEV1/FVC < 80%
FEV25-75% low.
PFT characteristics of restrictive: FVC < 80%
FEV1 low
FEV1/FVC normal! 80% or more
DLCO can be reduced if interstitial lung disease.

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