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Printed Case 1

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Title: Printed Case 1


1
Printed Case 1
  • Altered Mental Status in a Middle Aged Male
  • Helpful slides
  • Eugene Martin, Ph.D.

2
Patient Presentation - 1
  • As you read the patient presentation you might
    ask the following questions
  • What is the significance of the ? jugular venous
    pressure? What might it represent?
  • What is the significance of the patients mental
    status? What might it represent? Is it related to
    possible systemic disease? How might it relate?
    How would you rule it in or out?

3
Significance of the Increased Jugular Venous
Pressure.
  • JVP can be elevated in
  • heart failure
  • reduced right ventricular compliance (eg. RVH)
  • pericardial effusion, tamponade, constriction
    (Kussmaul's sign- JVP rises paradoxically in
    constrictive pericarditis (or sometimes in severe
    right- sided heart failure or tricuspid
    stenosis))
  • http//www.eboncall.org/CATs/2035.htm
  • Pericardial and pleural effusions sometimes
    represent manifestations of SLE

4
Patient Presentation - 2
  • Patient presents with slight dull heart and lung
    sounds and sharp chest pains. What do those
    symptoms suggest?

5
Pleural Effusion
  • 1 million pleural effusions in the United
    States each year.
  • Importance Incidental manifestations of
    cardiopulmonary diseases or symptomatic of
    inflammatory or malignant diseases
  • Most common symptom Dyspnea
  • Caused by distortion of the diaphragm and chest
    wall during respiration
  • Less common symptoms - mild, nonproductive cough
    or chest pain
  • Other symptoms suggest etiology
  • More severe cough or production of purulent or
    bloody sputum suggests an underlying pneumonia or
    endobronchial lesion.
  • Constant chest wall pain may reflect chest wall
    invasion by bronchogenic carcinoma or malignant
    mesothelioma.
  • Pleuritic chest pain suggests either pulmonary
    embolism or an inflammatory pleural process.
  • Systemic toxicity evidenced by fever, weight
    loss, and inanition suggests empyema

6
Pleural Effusion Cytospin
  • A Wright's stained cytospin preparation
  • What are the arrows pointing to?
  • Phagocytosed nuclear components of other cells in
    an antibody (ANA)-mediated process.

7
Our Patients Striking Lab Values
  • Pleural effusion, positive for LE cells
  • Urine, increased random and 24-hour protein
    concentration, the presence of blood, and a
    positive screen for cocaine metabolite
    (benzoylecgonine)
  • Whole blood, normocytic-normochromic anemia
  • Serum
  • ?? LD
  • ? BUN, creatinine, and AST levels,
  • ? ANA with diffusely staining pattern by IFA
  • ? dsDNA and Smith antibody tilers
  • ? decreased C3 and C4 levels
  • CSF, markedly increased total protein
    concentration.

8
Antinuclar antibodies
  • Staining Patterns originally thought to be
    indicative of specific disease
  • Homogenous
  • Nucleolar
  • Speckled
  • Centromere
  • Less important today because sensitivity and
    specificity than autoantibody testing
  • Lack of specifity Conditions associated w\
    positive ANA infections, pulm. Disease, liver
    disease, DM, MS, age, silicone gel implants,
    thyroid disease, medications
  • Significance of ANA in an asymptomatic
    individual?
  • Associated Conditions
  • SLE - gt99 ANA
  • Drug-induced lupus
  • Discoid lupus
  • Rheumatoid arthritis
  • Scleroderma 68 ANA
  • Mixed connect. Tiss. Dis
  • Sjögrens Syndrome
  • Polymyositis/dermatomyositis

9
ANA Profile
  • Abs to double-stranded DNA
  • Highly specific for SLE
  • 40-60 prevalence
  • Titers fluctuate w\ disease
  • Correlation w\ renal disease
  • Ab to Smith Ag
  • Highly specific, but in only 25 of SLE
  • Abs to histones
  • 70 of SLE patients
  • in drug-induced lupus as well as other
    connective tissue diseases
  • Abs to Ro (SSA)
  • Associated with Sjogrens syndrome, subacute
    cutaneous lupus, neonatal lupus, photosensitivity
    complement deficiency
  • Abs to LA (SSB)
  • Associated with Sjogrens syndrome, subacute
    cutaneous lupus neonatal lupus
  • Abs to single-stranded DNA
  • Freq. found in SLE BUT NOT SPECIFIC for SLE.
  • Found in other connect. Tissue diseases

10
Renal Status
  • Patient Urine, increased random and 24-hour
    protein concentration, the presence of blood, and
    a positive screen for cocaine metabolite

11
Renal Disease in SLE
  • Renal disease in SLE common (40)
  • At the time of SLE diagnosis 50 of patients have
    urinalysis abnormalities (proteinuria, hematuria,
    or pyuria)
  • Types of lupus nephritis
  • Mesangial
  • (10-20 of lupus nephritis)
  • Hematuria /or proteinuria mild
  • Hypertension uncommon
  • Renal function normal
  • Nephrotic syndrome unusual
  • Focal Proliferative
  • (10-20 of lupus nephritis)
  • Hematuria /or proteinuria in almost all patients
  • Some nephrotic syndrome present
  • Hypertension not uncommon
  • Elevated serum creatinine
  • Types of lupus nephritis (cont)
  • Diffuse Proliferative
  • Most common type of lupus neprhitis
  • Most severe
  • Hematuria /or proteinuria - all patients
  • Nephrotic syndrome common
  • Hypertension common
  • Renal insufficiency common
  • Hypocomplementemia usually present
  • Anti-ds-DNA abs usually present
  • Membranous
  • (10-20 of lupus nephritis)
  • Proteinuria all patients
  • Nephrotic syndrome common
  • Micro. Hematuria may be present
  • Hypertension may be present
  • Serum creatinine USUALLY normal, but may be
    mildly elevated.

12
Urine Protein Electropheresis
  • Electrophoretic pattern demonstrates
  • Non-selective proteinuria
  • Pattern is consistent with
  • Nephrotic Syndrome www.emedicine.com/ped/topic1564
    .htm
  • Nephrotic Syndrome is characterized by a urine
    protein concentration gt3.5 g/24 hr.
  • 50 of patients with SLE have nephritis
    characterized by functional abnormalities of the
    kidneys

13
Fluorescent Probe (400x)
  • Demonstration of patients antibody to mitotic
    figures
  • Patient's serum added to HEp-2 human epithelioid
    cells with mitotic figures Immunoconcepts, M)
    followed by the addition of a second antibody
    goat anti-human IgG (heavy and light chains)
    labeled with a fluorescent probe (FITC)

What are these?
14
Significance of Red Cell Casts
  • The presence of red cell casts is generally a
    clear indication of glomerular injury, or
    hemorrhage within the kidney.

15
Questions from the Hematology Lab Results
  • Patient demonstrates
  • 3.44 x 10-6 cells/ml normal 4.3-6.2 x 10-6
  • MCV 85.9 normal 82-105 fL
  • MCH 29.5 normal 28-34 pg
  • MCHC 34.3 normal 31-35 g/dL
  • Does this patient have anemia? If so, what kind?
    What is the significance of these values?
  • Normochromic, normocytic anemia
  • IF
  • Reticulocyte ?
  • Hemolytic disease
  • Acute blood loss
  • Reticulocyte normal
  • Malignancy
  • Myeloma
  • Chronic Disease
  • If this patient had a chronic blood loss for more
    than 6 months you would expect a hypochromic,
    microcytic anemia
  • The cases failed to tell you anything about the
    presence of reticulocytes, but SLE commonly
    presents with an Anemia of Chronic DIsease

16
Question from the Hematology Lab Data
  • Does this patient have anemia? If so, what kind?
  • Normochromic, normocytic anemia
  • Reticulocyte ?
  • Hemolytic disease
  • Acute blood loss
  • Reticulocyte normal
  • Malignancy
  • Myeloma
  • Chronic Disease
  • If this patient had a chronic blood loss for more
    than 6 months you would expect a hypochromic,
    microcytic anemia

17
Autopsy Finding
  • What happened?
  • A subdural hemorrhage
  • Darkened purplish area on the left side of the
    dura represents extravascular blood below the
    dura (dark arrows)
  • What might cause this?
  • Anti-phospholipid antibodies common in SLE ?
    prolonged PTT ? Bleeding disorders
  • Whats this?

18
SLE
  • Epidemiology
  • Prevalence of SLE worldwide is 4-250 per 100,000.
  • Incidence is most frequent in women age 15-25
    years.
  • 9x as many females as males
  • Blacks and Asians are affected more often than
    other races
  • Broad age category between 10 ?50
  • Course of disease ranges from mild ? severe/fatal
  • lt50 have lupus nephritis
  • 25 have neurologic symptoms
  • Clinical Presentation
  • Varies between different patients and within a
    patient Constitutional symptoms (fatigue and
    fever) are common.
  • Vast majority have arthralgia, mostly of the
    hands.
  • one-half of the patients have cutaneous
    features, such as malar rash and discoid lupus as
    well as photosensitivity.
  • 30 have oral ulcerations.
  • 50 have nephropathy from mild proteinuria and
    microscopical hematuria to end-stage renal
    failure.
  • 20-40 of the patients have pleurisy.
  • Pericarditis is somewhat more uncommon than
    pleuritis. T-wave changes in the
    electrocardiogram (ECG) are usual.
  • Depression and headache are the most common of
    the neuropsychiatric symptoms. Grand-mal seizures
    and organic psychoses are rare. A peripheral
    neuropathy is observed in about 10 of the
    patients
  • 10 get a thromboembolic or hemorrhagic
    complication of the brain.
  • The lymph nodes may enlarge especially in active
    disease.

19
SLE COMMON SYMPTOMS
  • fever
  • fatigue
  • general discomfort, uneasiness or ill feeling
    (malaise)
  • weight loss
  • v skin rash
  • v "butterfly" rash
  • v sunlight aggravates skin rash
  • v sensitivity to sunlight
  • v joint pain and swelling virtually all
  • arthritis
  • swollen glands
  • muscle aches
  • nausea and vomiting
  • v pleuritic chest pain
  • v seizures
  • ? psychosis
  • v - Our patient exhibited

20
Laboratory Correlations
  • ANA antibodies
  • 97 of patients with SLE,
  • 30 to 40 of patients with RA,
  • 70 of patients with scleroderma.
  • dsDNA antibodies
  • 70 to 80 of patients with SLE
  • 15 of patients with RA
  • 30 of patients with scleroderma.
  • Anti-Sm antibodies
  • High specificity, low sensitivity for SLE
  • Immunfluorescent Patterns
  • Rim, diffuse, and speckled patterns - SLE
  • nucleolar scleroderma
  • centromere - CREST (Calcinosis cutis-Raynaud's
    phenomenon-Esophogeal dysfunction-Sclerodactyly-Te
    langiectasia) syndrome

21
Less Common Symptoms
  • Blood in the urine
  • Coughing up blood
  • Nosebleed - symptom
  • Swallowing difficulty
  • Skin color is patchy
  • Red spots on skin
  • Abdominal pain
  • Visual disturbance
  • Fingers that change color upon pressure or in the
    cold (Raynaud's phenomenon), numbness and
    tingling
  • Mouth sores
  • Hair loss

22
Blood disorders effect up to 85 of those with SLE
  • Venous or arterial blood clots can form and are
    associated with strokes and pulmonary embolism,
    or pregnancy loss.
  • Predisposition to blood clots, or less commonly
    bleeding, occurs due to antibodies against lipids
    involved in blood clotting and is referred to as
    anti-phospholipid antibody syndrome (APS). (See
    Lupus anticoagulant.)
  • Often platelets are decreased which may cause
    bleeding or clotting problems.
  • Anemia of chronic disease often develops at some
    point in the course SLE.

23
Diagnosis of SLE
  • v antinuclear antibody (ANA) panel including
    anti-DNA and anti-Smith antibodies, with the
    latter two tests generally positive in lupus
    alone
  • v characteristic skin rash or lesions
  • v chest X-ray showing pleuritis or pericarditis
  • v Hematologic abnormalities hemolytic anemia. 2
    or more instances of lymphopenia. Anemia of
    chronic disease.
  • listening to the chest with a stethoscope to
    reveal heart friction rub or pleural friction rub
  • v urinalysis to show blood, casts, or protein in
    the urine
  • v CBC showing a decrease in some cell types
  • v kidney biopsy demonstrates glomerulonephritis
  • v neurological examination demonstrates seizure
    activity or psychosis
  • v Our patient demostrates.

24
Lab Values Frequently Affected in SLE
  • WBC count
  • Serum globulin electrophoresis
  • Rheumatoid factor
  • Protein, urine
  • Protein electrophoresis - serum
  • Mononucleosis spot test
  • ESR
  • cryoglobulins
  • Coombs' test, direct
  • complement component 3 (C3)
  • complement
  • Anti-thyroid microsomal antibody
  • Anti-thyroglobulin antibody
  • Anti-mitochondrial antibody
  • Anti-smooth muscle antibody

25
Complications of SLE
  • Infection
  • Renal failure
  • Thrombocytopenia
  • Hemolytic anemia
  • Myocarditis
  • Seizures

26
Treatment
  • Multiple manifestations with variable severity,
    determine individual treatment
  • Mild disease (rash, headaches, fever, arthritis,
    pleurisy, pericarditis) requires little therapy.
  • Nonsteroidal anti-inflammatory medications
    (NSAIDS) - arthritis and pleurisy.
  • Corticosteroid creams are used to treat skin
    rashes.
  • Antimalarial drugs (hydroxychloroquine) and low
    dose corticosteroids are sometimes used for skin
    and arthritis symptoms.
  • Severe disease (hemolytic anemia, extensive
    heart or lung involvement, kidney disease,
    central nervous system involvement)
  • Corticosteroid therapy or medications to suppress
    the immune system
  • Cytotoxic drugs for patients people who do not
    have a good response to corticosteroids or who
    are dependent on high doses of corticosteroids.

27
Printed Case 2
  • 38 year old white man seen by his family
    physician two months ago for a chief complaint of
    fever, fatigue, malaise, and pain localized over
    his nose and maxillary sinuses

28
Chest X-ray
  • What do you see?
  • Right mid-lung field lesion (about 4cm in
    diameter) - Tomogram Lobulated Central Cavity
    (Below)
  • Left mid-lung field lesion (about 1.5cm in
    diameter),
  • Ill-defined area of increased left-based
    density.

What do you see?
29
Paranasal sinus x-rays
  • What do you see?
  • Mucosal thickening left antrum
  • Mass - upper portion nose, without bone
    destruction.

30
Ulcer adjacent to the middle turbinate
  • Giant cells
  • Necrotizing vasculitis

31
Differential Dx. Hemoptysis
  • Intrinsic benign and malignant tumors of the
    larynx, pharynx, trachea, bronchi, and lungs
  • Granulomatous disease (i.e. Tb. fungal,
    parasitic, Wegener's)
  • Bronchogenic cyst
  • Broncholithiasis
  • Bronchiectesis
  • Erosion of trachea or bronchi by extrinsic tumor
  • Acute chronic bronchitis
  • Pulmonary abscess pneumonia
  • Hemosiderosis
  • Goodpasture's syndrome trauma
  • Left ventricular failure pulmonary
    embolism/infarction
  • Pulmonary arteriovenous fistula
  • Primary pulmonary hypertension Thrombocytopenia
  • DIC
  • Anticoagulant therapy, etc.

32
Wegeners Granulomatosis
  • Wegeners Triad
  • Acute necrotizing granulomas of upper/lower
    respiratory tract.
  • Focal necrotizing or granulomatous vasculitis of
    small to medium-sized vessels, most prominent in
    lungs and upper airways.
  • Focal or necrotizing (often crescentic)
    glomerulitis.
  • Necrosis may cavitate-diff dx includes TB,
    fungus.
  • Common normochromic, normocytic anemia and
    hypergammaglobulinemia
  • c-ANCA in 90 patients with active generalized
    disease.
  • Peak incidence is in the 30-40s (Malefemale
    32).
  • gt 50 ocular inflammation
  • -Persistent pneumonitis with bilateral
    nodular/cavitary infiltrates (95)
  • -Chronic sinusitis (90)
  • -Nasopharyngeal ulcerations (75)
  • -Renal disease (80) http//www.emedicine.com/emer
    g/topic219.htm
  • -Also, rash, myalgias, arthralgias, neuritis,
    fever.

33
  • http//www.emedicine.com/emerg/topic219.htm

34
Characterize the slides
  • Necrotizing Vasculitis
  • Giant Cells
  • Top HE 40X
  • Bottom HE 80X

35
ANCA
p-ANCA is perinuclear against MPO.
C-ANCA shows cytoplasmic localization
  • ANCA - heterogenous autoantibodies against
    enzymes in primary granules of neutrophils though
    also found in lysosomes of monocytes and
    endothelial cells
  • C-ANCA Most common against serine protease in
    neutrophils (proteinase 3)
  • p-ANCA is perinuclear against MPO.

36
C-ANCA
  • Very sensitive for diagnosis of Wegeners
    granulomatosis
  • Sensitivity/Specificity
  • Active disease sensitivity gt 90
  • Organ limited disease sensitivity 60
  • Remission sensitivity 40
  • Specificity 90
  • A positive c-anca in a patient who exhibits class
    SS of Wegeners may obviate biopsy

37
P-ANCA
  • Present 70 of Churg-Strauss syndrome and
    microscopic polyangitis patients however test is
    neither sensitive or specific enough to establish
    a dx. (biopsy recommended)
  • Maybe in
  • Wegeners
  • RA
  • SLE
  • Goodpastures syndrome
  • Ulcerative colitis
  • Autoimmune hepatitis
  • Etc.
  • Titer association w\ disease state not tight ?
    ? patient monitoring
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