Post on 04-Jan-2016
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APS in Lebanon.
Imad Uthman, MD, MPHImad Uthman, MD, MPHProfessor of Clinical MedicineProfessor of Clinical MedicineHead, Division of RheumatologyHead, Division of RheumatologyAmerican University of Beirut Medical CenterAmerican University of Beirut Medical CenterBeirut, LebanonBeirut, Lebanon
Study Of The Clinical And Serological Characteristics And Long-term Evolution of APS Patients
At The American University of Beirut Medical Center (AUBMC).
The Lebanese APS Study Group.
Purpose: – To evaluate prospectively the clinical
manifestations and the long-term evolution of the APS patients seen at AUBMC and, in the future, expand this to other centers in the country.
The Lebanese APS Study Group.
Methods: – All patients included have fulfilled at least
one clinical criterion and one laboratory criterion.
– A standardized questionnaire was used to collect demographic data, risk factors for thrombosis, coexisting autoimmune disorders, and specific morbidities due to APS, and data was stored into a registry.
The Lebanese APS Study Group.
Results: – 200 patients logged into the registry so
far.– Cases were identified from the lab records
(positive ACA and/or LAC tests)
Demographic
Lebanon Europe*
Female: 144 (72%) 820 (82%)
Male 46 (28%) 180 (18%)
Cervera R, et al on behalf of the "Euro-Phospholipid Project Group”: Antiphospholipid syndrome: Clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients. Arthritis Rheum 2002; 46: 1019-1027
Underlying Conditions
Lebanon Europe*
SLE 78 (39%) 362 (36.2%)
Primary APS 70 (35%) 531 (53.1%)
RA 4 (2%) 18 (1.8%)
Systemic Sclerosis 4 (2%) 7 (0.7%)
Sjogren’s Syndrome
2 (1%) 22 (2.2%)
Lupus Like Syndrome
2 (1%) 50 (5.0%)
*Cervera R, et al : Arthritis Rheum 2002; 46: 1019-1027
Neurological Manifestations
Lebanon Europe
Stroke 34 (17%) 198 (19.8%)
Transient Ischemic Attack
16 (8%) 111 (11.1%)
Epilepsy 16 (8%) 70 (7.0%)
Migraine 14 (7%) 202 (20.2%)
Cerebellar ataxia: 6 (3%) 7 (0.7%)
Cerebral venous thrombosis:
4 (2%) 7 (0.7%)
Vertigo 4 (2% )
Cardiac Manifestations
Lebanon Europe
Angina 6 (3%) 27 (2.7%)
Valve dysfunction 3 (1.5%) 116 (11.6%)
Coronary artery bypass graft
2 (1%) 11 (1.1%)
Chronic cardiomyopathy
2 (1%) 29 (2.9%)
Myocardial infarction 55 (5.5%)
Intestinal Manifestations
Lebanon Europe
Splenic infarcts: 10 (5%) 11 (1.1%)
Pancreatic infarction:
6 (3%) 5 (0.5%)
GI ischemia: 6 (3%) 15 (1.5%)
Liver infarctions: 4 (2%)
Budd Chiari syndrome
1 (0.5%) 7 (0.7%)
Addison’s Disease: 1 (0.5%) 4 (0.4%)
Pulmonary Manifestations
Lebanon Europe
Pulmonary embolism & infarction
15 (7.5%)
141 (14.1%)
ARDS 3 (1.5%)
7 (0.7%)
Osteoarticular Manifestations
Lebanon Europe
Arthralgias: 28 (14%) 387 (38.7%)
Arthritis: 10 (5%) 271 (27.1%)
Avascular necrosis:
8 (4%) 24 (2.4%)
Obstetric Manifestations
Lebanon Europe
Preeclampsia/ eclampsia:
4 (2%) 56 (9.5%)/
26 (4.4%)
Abruptio Placenta:
4 (2%) 12 (2.0%)
Fetal Manifestations
Lebanon Europe
pregnancies 296 1580
Early Fetal Loss (<10 weeks)
56 (19%) 560 (35.4%)
Late Fetal Loss (>10 weeks):
20 (6.75%) 267 (16.9%)
Live birth 200 (67%) 753 (47.7%)
Premature birth, no. premature/no. live births
12/200 (6%) 80/753 (10.6%)
Thrombotic Manifestations
Lebanon Europe
LE DVT 56 (28%) 389 (38.9%)
LE superficial phlebitis
6 (3%) 117(11.7%)
Renal Manifestations
Lebanon Europe
Renal vein thrombosis
3 (1.5%)
Renal infarction 2 (1%)
Renal Manifestations
27 (2.7%)
THE LEBANESE APS STUDY GROUP
Results: – In decreasing frequency, the most common
complications were: • DVT
• Obstetric problems
• Stroke
• TIA
• Seizures
• Migraines
• Splenic infarcts
THE LEBANESE APS STUDY GROUP
Conclusion: – DVT and strokes are the most common
non-obstetric complications of APS in Lebanon.
– Many patients suffer from more than one complication, such as DVT or ischemic stroke.
Nightmare On Bliss StreetCatastrophic Cerebral APS
Case Presentation
Mrs. HM 61 year old female presented to ER on March 8, 09 with acute onset of dysarthria and left cheek numbness.
One month before she had recurrent episodes of vertigo and unsteadiness, was told she had ear problems.
Background
Hypothyroid and dyslipidemic.Smoker Married 3 childrenNo Hx of abortion, fetal loss, or PreclampsiaHx of premature delivery twice (at 32,30wks)No Hx of DVT.
Physical Exam
BP: 200/100.Dysarthria.Right ptosis. Cerebellar signs: Normal Gait: Normal Sensory Exam: Normal
MRI Brain: 9 March 09
Impression: – Multiple acute lacunar infarcts in
watershed distribution in both centrum semi-ovale as well as in the left precentral gyrus.
– Subacute infarct in the left occipital area.
MRI Brain: 9 March 09
MRI Brain: 9 March 09
MRI Brain: 9 March 09
MRI Brain: 9 March 09
Assessment
Neurology: R/O Vasculitis
Rheumatology: R/O APS
Laboratory Evaluation
Lupus anticoagulant: Positive
ACA IgG: 96.4 GPLu/mL ACA IgM: <7 MPLu/mL
Anti ß2-Glycoprotein I IgG: >200 U/ml Anti ß2-Glycoprotein I IgM: <2 U/ml
Laboratory Evaluation
ANA: NegativeAnti ds-DNA: NegativeC-ANCA: Negative
Course in Hospital
Started on Heparin, Plavix.Next day ( March 10) marked
improvement in speech
Course in Hospital
March 11:– Worsening dysarthria.– PTT was in the therapeutic range.– Started on Prednisone 1mg/kg by the
neurology team (for suspicion of CNS vasculitis).
March 14 PM:– Worsening of numbness and weakness in the
right upper extremity + had tongue numbness – INR 2.6– BP: 175/110 – Solu-Medrol 250 mg IV given – Restarted on IV Heparin
Course in Hospital
15 March Evening:– Acute right sided weakness 0/5 motor power
Rt side + Aphasia.
Course in Hospital
MRI Brain: 15 March 09
Impression: – Acute infarcts in the left parietal and frontal
lobes and in the watershed territories bilaterally more so on the left.
– Small focus of hemorrhage in the left parietal lobe.
MRI Brain: 15 March 09
MRI Brain: 15 March 09
MRI Brain: 15 March 09
MRI Brain: 15 March 09
16 March:– 12 PM: Unconscious, seizure like activity.
– Acute respiratory distress: mechanical ventilation.
– Physical Exam: Pupils mid sized dilated Absent corneal reflexAbsent Oculocephalic reflex Decerebrate posturing Bilateral positive Babinski
Course in Hospital
CT Brain: 16 March 09
Impression: – Hemorrhagic transformation in the left frontal
lobe infarct with significant surrounding edema, mass effect, trans-tentorial and uncal herniations with brain stem infarct.
CT Brain: 16 March 09
CT Brain: 16 March 09
CT Brain: 16 March 09
CT Brain: 16 March 09
17 March (one week after her initial presentation):– Declared brain dead, family decided to stop
mechanical ventilation.
Course in Hospital
Nightmare on Bliss Street
The Autopsy
Brain Gross Pathology
Soft, edematous Lt Sub-falcial
herniationUncal herniation and
necrosis.
Terminal Massive ICH
Recent large intracerebral hemorrhage, left frontal and parietal lobes.
Fibrin thrombi within a vessel
Free-floating small fibrin thrombi within rare arterioles and venules in cortex, leptomeninges, and white matter hemorrhage.
LF WM, recent hemorrhage, fibrin
Small recent infarcts, Left Occipital Cortex
Focal Perivascular lymphocytic infiltration.
Microangiitis, vasa vasorum
Perivascular lymphocytic infiltrates, multifocal, involving small vessels of vasa vasorum, right and left carotid arteries, consistent with small vessel angiitis.
Summary
This is the first large series of APS patients from Lebanon.
Although testing for APS is available in major hospitals in the country, the condition is still largely unrecognized by the medical community.
Further testing including genetic studies are planned in our cohort.
Thank You