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• Generalized weakness
– Persisted and progressed that
patient needed help in order
to ambulate
HPI:
51 weeks prior • Anorexia
to • Skin sores in the anterior
admission thorax
• Wounds on her breast around
the areolar region
• Recurrence of headache
• Persistence of joint and
muscle pains and
generalized weakness
• Prompted consultation at
Medical City
– Tested for visual problems = (-)
– Asked to consult psychiatrist
=did not comply
HPI:
50 weeks prior • Continuous fever = 400C
to – Persisted for a week
admission
• Bluish discoloration of fingers
• Prompted another
consultation at Medical City
• No history of surgery
• HEENT:
– Head: No history of head injury.
– Eyes: No visual dysfunction or lacrimation. No
itchiness, pain or recent changes in vision.
– Ears: Hearing good. No tinnitus, vertigo,
infections.
– Nose, sinuses: No hay fever, sinus trouble.
– Throat: No soreness or tonsilitis.
Review of Systems
• Neck: Has stiff neck. No lumps, goiter, pain. No
swollen glands
• Nails: No clubbing
PHYSICAL
EXAMINATION
ON ADMISSION ON ACTUAL EXAMINATION
General Survey General Survey
• Conscious, coherent, • Conscious, coherent,
ambulatory and in oriented as to time,
cardiorespiratory place and person, not
in cardio-respiratory
distress distress
• Height- 5’6’’
Weight- 65 kg
PHYSICAL
EXAMINATION
ON ADMISSION ON ACTUAL EXAMINATION
General Survey General Survey
• non-erythematous
macular lesions less
than 1cm in the anterior
thorax near the neck
Malar Rash
Source: http://www.emedicine.com/med/images/329097-
332244-5270.jpg
PHYSICAL
EXAMINATION
ON ADMISSION ON ACTUAL EXAMINATION
HEENT HEENT
• Pale palpebral • Pale palpebral
conjuctiva, anicteric conjuctiva, anicteric
sclera sclera
Respiratory Respiratory
• Symmetrical chest
expansion • Symmetrical chest
• no retractions expansion
• no retractions
• Resonant
• decreased tactile and • decreased tactile and
vocal fremiti
• decreased breath vocal fremiti in the
sounds on the left T6- left posterior lower
T7 down lung region
• (+) crackles on left • fine rales heard
lung field
PHYSICAL
EXAMINATION
ON ADMISSION ON ACTUAL EXAMINATION
Cardiovascular Cardiovascular
• Adynamic precordium, • JVP 3.0cm above the
apex beat 5th LICS sternal angle at 300
MCL (apex: S1>S2; • CAP has rapid
base: S2>S1), no upstroke gradual
murmurs downstroke
• Adynamic precordium,
apex beat at the 5th
LICS, MCL.
• Apex: Loud S1
followed by soft S2;
base: Soft S1 followed
by loud S2, splits at
inspiration , no
murmurs, no heaves,
thrills, or lifts.
PHYSICAL
EXAMINATION
ON ADMISSION ON ACTUAL EXAMINATION
Abdominal Abdominal
• Flabby abdomen • Abdomen is distended
and shiny with silvery
white striae
• normoactive bowel • normoactive bowel
sounds sounds, no bruits
Extremities Extremities
• Pulses full and equal, • (+) edema
(+) edema, (-)
cyanosis
PHYSICAL
EXAMINATION
Central and Peripheral Pulses
(0)=absent
(+)=decreased
(++)=normal
(+++)=hyperactive
(Br)=bruit
PULSE CAROTID BRACHIAL RADIAL FEMORAL POPLITEAL DOR PEDIS POST TIBIAL
RIGHT ++ ++ ++ ++ ++ ++ ++
LEFT ++ ++ ++ ++ ++ ++ ++
PHYSICAL
EXAMINATION
ON ACTUAL EXAMINATION
Musculoskeletal
• Temporomandibular joint: no skin changes,
abnormal contours or deformities; no swelling or
tenderness, good movement
Upper extremities
• Shoulder joint: no skin changes and deformities,
symmetrical, no swelling and tenderness, no
limitation in motion
• Elbow joint: no skin changes and deformities, no
tenderness and thickening, slight limitation in
motion due to edema, right elbow has an IV so
could not be moved
PHYSICAL
EXAMINATION
ON ACTUAL EXAMINATION
Upper extremities
• Forearm, wrist and hand: edematous, bulky
hands; no redness, nodules, no
metacarpophalangeal tenderness; no
interphalangeal tenderness; right hand unable to
make a fist and has limited extension and flexion;
left hand has limited extension and slightly limited
flexion.
Lower Extremities
• Hip joint: no skin changes, abnormal contours
and deformity; no swelling and tenderness; no
limitation in movement
PHYSICAL
EXAMINATION
ON ACTUAL EXAMINATION
Lower Extremities
• Knee joint: no skin changes, abnormal contours
and deformity; no muscle atrophies and
thickening; slight swelling due to edema and no
tenderness in the patellar region; no limitation in
movement, no instabilities; (-) bulge sign, (-)
balloon sign
Data from Tan EM, et al. The Revised Criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 25:1271, 1982; and Hochberg, MC: Updating
the American College of Rheumatology revised criteria for he classification of systemic lupus eryhtematosus. Arthritis Rheum 40: 1735, 1997
1997 REVISED CRITERIA FOR CLASSIFICATION
OF
SYSTEMIC LUPUS ERYTHEMATOUS
• 11 criteria
• SLE
– 4 or more are present
– serially or simultaneuosly, during any interval of
observation
• Patient
– arthritis
– oral ulcers
– malar rash
– renal disorder
– hematologic disorder
Definition of Terms
• Rheumatology
– rheuma – that which flows as a river or
stream
– encompasses the autoimmune diseases,
arthritis, and musculoskeletal
conditions. While rheumatologists are
generally thought to be specialists
dedicated to rheumatoid arthritis, lupus
and scleroderma, we also care for
patients with a wide array of systemic,
inflammatory, autoimmune diseases as
well as very common musculoskeletal
disorders
Source: (e.g., osteoarthritis and
http://en.wikipedia.org/wiki/Rheumatology
osteoporosis) and sports-related
http://www.rheumatology.org/students/faq.asp injuries.
(American
College of Rheumatology)
Definition of Terms
• Rheumatism
– non-specific term used to describe any
painful disorder affecting the loco-motor
system including joints, muscles,
connective tissues, soft tissues around
the joints and bones
– A term used for acute and chronic
conditions marked by inflammation,
muscle soreness and stiffness, and pain
in http://en.wikipedia.org/wiki/Rheumatology
Source: joints and associated structures
Pathophysiology of SLE
Autoimmune Diseases
• autoantibodies react against self-antigens
From Robbins and Cotran Pathologic Basis of Disease 7th ed. p 223
Self-tolerance
• lack of responsiveness to an individual’s own
antigens
• 2 postulated mechanisms
– Central Tolerance
• clonal deletions of self-reactive T and B cell clones occur during
their maturation
• some may still reach the periphery
– Peripheral Tolerance
• anergy - permanent inactivation of lymphocytes
• deletion by apoptosis
• suppression by regulatory T cells
• antigen sequestration
• environmental factors
– infection
• co-stimulators on APCs are indcued loss of anergy
• molecular mimicry .
• Implicated factors
– Genetic
– Environmental
– Hormonal
– Primary abnormality in the immune system
From Robbins and Cotran Pathologic Basis of Disease 7th ed. and Harrison’s Principles of
Internal Medicine 16th ed.
Genetic Factors
• HLA-DQ genes in chromosome 6
– C4 deficiency
• loss of B-cell tolerance
– C1q deficiency
• failure of phagocytosis of apoptotic cells
• nuclear components of such cells may
induce an immune response
• highest genetic risk
From Robbins and Cotran Pathologic Basis of Disease 7th ed. and Harrison’s Principles of
Internal Medicine 16th ed.
Environmental Factors
• hydralazine, procainamide, and
D-penicillamine lupus-like
response
• UV exposure
– exacerbations
– stimulates keratinocytes to
produce IL-1 apoptosis
– apoptotic cells may become
antigenic
From Robbins and Cotran Pathologic Basis of Disease 7th ed. and Harrison’s Principles
of Internal Medicine 16th ed.
Hormonal Factors
• OC pills, HRT
– 2x risk of developing SLE
• Estradiol
– bind to receptors on T cells and B cells
– increased activation and survival
extended immune response
From Robbins and Cotran Pathologic Basis of Disease 7th ed. and Harrison’s Principles of
Internal Medicine 16th ed.
Autoantibodies
• ANAs can be grouped into:
– Antibodies to DNA
– Antibodies to histones
– Red cells
– Platelets
– Lymphocytes
• 3 phases
– Determinants if deposition
will lead to a disease state
• size of complex: intermediate
or small
• Intact mononuclear
phagocyte system
– Favored sites:
• renal glomeruli
• joints
• skin
• heart
• serosal surfaces
• small blood vessels
– microthrombi formation
• Vasculitis
• Glomerulonephritis
• Arthritis
contour
Radiology
• Acute lupus
pneumonitis
– Increased opacity
and poorly defined
markings in the mid
and lower portions of
the chest - (R) x-ray
film
Pharmacologic
GLUCOCORTICOIDS
• Indications:
• life-threatening manifestations of SLE, such
as glomerulonephritis, CNS involvement,
thrombocytopenia, and hemolytic anemia
• debilitating manifestations of SLE (fatigue,
rash) that are unresponsive to conservative
therapy
UTI Ciprofloxacin
Pulmonary Infiltration Corticosteroids
www.uic.edu/classes/pmpr/pmpr652/final/stevens/sle.html#SLE
• Adequate sleep and avoidance of fatigue
• Regular exercise
• Education about lupus and self-care
• Avoid smoking
• Eating a healthy balanced diet
• Salt restriction
• Developing a support system of family, friends,
and health professionals
• For patients with photosensitive rashes use
protective clothing, such as a hat and long
sleeves, and avoidance of sun exposure are
recommended to prevent flareups
Thank you!
Kidneys
• Nephritis
– Immune complex deposition in renal
structures:
• Glomeruli
• Tubular & peritubular capillary basement
membranes
• Thrombosis
– Glomeruli
– Extraglomerular vasculature
• Hypercoagulable state