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Three cases of Behcet`s disease with vascular involvement

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1 Three cases of Behcet`s disease with vascular involvement
IN THE NAME OF GOD Three cases of Behcet`s disease with vascular involvement Gholamrezapoor, MD, resident of internal medicine & Sasan Fallahi, MD, rheumatologist, Kerman University of Medical Sciences

2 CASE PRESENTATION

3 FIRST CASE A 48 years old female

4 HISTORY Chief complaint Pain and swelling of the left lower limb
Present illness Patient’s problem has been started from four weeks ago, initially she had pain and subsequently swelling of the left lower limb. Swelling appeared initially in distal side of leg and then extended to proximal side of the left thigh. She also had history of fever, especially at the evening. No dyspnea, chest pain and hemoptysis.

5 HISTORY Past History she did not have any previous history of known illness, except admission for PID, 7 years ago. She also, had an abortion. No history of recent surgery, trauma, bed ridden or traveling. She used Prednisolone(5mg daily),Omeprazol, Doxepin and Loratadin since 4 weeks ago, but she did not used OCP.

6 HISTORY Family History There was no significant point.
Personal and Social History She was not smoker and opium addict.

7 HISTORY Review of Systems Oral painful lesions Genital painful lesions
Since 8 years ago Interval 20 days Duration days At the time of physical examination, the lesion was present. Genital painful lesions Since 4 years ago Interval several months Duration 5-7 days At the time of physical examination, the lesion was not present. Dysuria When genital lesion was present

8 HISTORY Review of Systems
Red eye, tearing and Pain in left eye since 20 years ago intermittently. No history of visual loss Skin lesion in both lower limbs since 15 days ago No history of headache and seizure No history of GI problems such as abdominal pain or diarrhea No history of arthralgia or arthritis

9 PHYSICAL EXAMINATION General Survey
Patient is a middle age female, awake and oriented, without any distress, she was thin and pale. Vital Signs PR:80 RR:15 BP:90/60 AxilaryT:37

10 PHYSICAL EXAMINATION Head & Neck
-Mild conjunctivitis and tearing in left eye was noted(red eye). -Aphthous lesions in right and left side of tongue were noted -Trachea and thyroid were normal - No adenopathy Chest & Cardiac & Axillary NL Abdomen Prominent veins were visible in epigastric zone, with flow from down to up. There was no distention, tenderness, hepatomegaly, splenomegaly and ascites.

11 PHYSICAL EXAMINATION Upper extremities -No skin lesion
-Bilateral radial arteries pulsation were normal and symmetric . -Active and passive motion of joints were normal. Force of proximal and distal muscles were near to normal for her sex and age

12 PHYSICAL EXAMINATION Lower extremities
-Swelling and pitting edema in left lower limb, specially in foot, ankle and distal side of leg -Size difference between circumference of two legs was about 2.5cm. -Red brownish colored nodule with tenderness (1.5 * 1.5 cm ) on lateral side and anterior surface of the left leg compatible with erythema nodosum -Dorsalis pedis and posterior tibialis arteries pulsation in left side were palpable but weaker than right side. Active and passive motion of joints were normal. Force of proximal and distal muscles were near to normal.

13 DUPPLER SONOGRAPHY Thrombosis in CFV and SFV were noted.
Venous thrombosis was extended to the left iliac vein and IVC. SMA, SMV, Portal vein and hepatic arteries were normal.

14 DIANOSIS DVT

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16 LABORATORY TEST CBC WBC:7500 RBC:3790000 HG:9.2 HCT:31.3 MCV:82.6
MCH:24.3 MCHC:24.4 Plat:360000 ESR:104 CRP:+3 RF: Neg

17 LABORATORY TEST Biochemistry BS:86 Urea:15 Creat:0.89 AST:18 ALT:12
Al Ph:255 Bil Total:0.6 Bil Direct:0.16 LDH:435 Electrolyte Na:135 K:3.9 Ca:8.5 P:4.5 Coagulative PTT:41 PT:15 INR:1

18 LABORATORY TEST PBS Hypochromic:+1 Anisocytosis:Mild
Poychilocytosis:+1 Ovalucytosis:Mild Teardrop:Mild Helmet:Mild Urine analysis SG:1015 PH:7 Others: NL

19 ECHOCARDIOGRAPHY EF:60% PAP:NL There was no abnormal finding.

20 ABDOMINOPELVICE SONOGRAPHY
Liver, biliary tract, pancreas and urinary tract were normal

21 GYNECOLOGICAL CONSULT
No aphthous, active lesion or scar in genital area

22 OPHTHALMOLOGICAL CONSULT
There was naso-lacrimal duct stenosis in left eye. No evidence of uveitis or retinal vasculitis

23

24 SECOND CASE A 36 years old male

25 HISTORY Chief complaint
Pain and swelling of the right lower limb since two weeks ago Present illness -Swelling has been appeared initially in right foot and then extended to the right leg and thigh. -No fever, dyspnea, chest pain and hemoptysis

26 HISTORY Past History -known case of DM since six months, ago
-No history of recent surgery, trauma or bed rest, but he had a trip by bus , 45 days ago.

27 HISTORY Family History No significant point.
Personal and Social History No smoking and opium addiction

28 HISTORY Review of Systems Oral painful lesions Genital painful lesions
The first time appeared at and continued for 2 weeks and then disappeared for 5 years Further started from 1380 Interval 20 days Duration about 15 days At the time of physical examination, the lesion was present. Genital painful lesions The first time appeared at 1380 And reoccurred several times . Duration 3-5 days At the time of physical examination, there was no lesion or scar.

29 HISTORY Review of Systems
-45 days, ago a few skin pustular lesions appeared on right leg. only two small brownish papule remained. -No history of erythema nodosum -Swelling of right testis one month, ago -No history of visual loss, red eye and ocular pain -No history of headache and seizure -No history of GI problems such as abdominal pain or diarrhea -No history of arthralgia or arthritis

30 PHYSICAL EXAMINATION Patient is a young male, awake and oriented, without any distress. Vital Signs PR:84 RR: BP:120/ AxillaryT:37.4

31 PHYSICAL EXAMINATION Head & Neck
-An aphthous lesion in anterior side of tongue was noted. -Trachea and thyroid were normal. -No adenopathy Chest & Cardiac & Axillary NL Abdomen -No distention, tenderness, hepatomegaly, splenomegaly and ascites

32 PHYSICAL EXAMINATION Upper extremities -No skin lesion
-Bilateral radial arteries pulsation were normal and symmetric. -Active and passive motion of joints were normal. -Force of proximal and distal muscles were normal.

33 PHYSICAL EXAMINATION Lower extremities
-Swelling and pitting edema in right lower limb, specially in foot, ankle and distal side of leg -Size difference between circumference of two legs was about 1.5cm. -Two skin lesions (brownish colored pigmentation with 3 * 3mm in size )were visible on lateral side of the right leg (scars of pseudofolliculitis). Bilateral dorsalis pedis and posterior tibialis arteries pulsation were palpable, normal and symmetric. -Active and passive motion of joints were normal. -Force of proximal and distal muscles were normal .

34 DUPPLER SONOGRAPHY Thrombosis in popliteal vein
CFV and SFV were normal. SMA, SMV, portal vein and IVC were normal.

35 DIAGNOSIS DVT

36

37 LABORATORY TEST CBC WBC:7400 RBC:5180000 HG:14.3 HCT:45 MCV:87 MCH:28
MCHC:32.1 Plat:374000 Biochemistry BS:207 Urea:31 Creat:1 AST:14 ALT:13 Al Ph:234 Bil Total:0.75 Bil Direct:0.16 Uric acid:4.8

38 LABORATORY TEST Electrolyte Na:137 K:4.2 Ca:9.2 P:3.5 Coagulative
PTT:31 PT:13 INR:1.1

39 Pathergy test

40 THIRD CASE 34 years old male

41 HISTORY -Diplopia and visual loss since Farvardin, 1390 due to thrombosis of cerebral venous sinuses, increase of ICP and optic disk atrophy. -8 months, ago he had DVT in left lower limb for which Warfarin started. -Post prandial abdominal pain 6 months, ago

42 HISTORY -Abdomino-pelvice CT scan: vascular aneurysm was suspected. -CT Angiography showed aneurysm in abdominal aorta and right common iliac artery. -Operation was done and Prednisolone,60mg daily and Cyclophosphamide, monthly were started.

43 HISTORY Review of system -Oral aphthous since the age of eight
-No history of genital lesions -No history of erythema nodozum or pseudofolliculitis -No history of arthritis

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53 Positive pathergy test
THE FIRST CASE Oral aphtous + Erythema nodosum DVT Positive pathergy test Behcet’s disease

54 THE SECOND CASE Oral aphtous + Pseudofolliculitis Lesions DVT Positive pathergy test Behcet’s disease

55 THE THIRD CASE Oral aphtous + Arterial aneurysm Positive pathergy test
Increased ICP, Cerebral venous sinus thrombosis and lower limb DVT Arterial aneurysm Positive pathergy test Behcet’s disease

56 VIRCHOW'S TRIAD Proposes that VTE occurs as a result of
Alterations in blood flow (stasis) Vascular endothelial injury Alterations in the constituents of the blood (inherited or acquired hypercoagulable state)

57 RISK FACTORS FOR VENOUS THROMBOSIS
Inherited thrombophilia Factor V Leiden mutation Prothrombin gene mutation Protein S deficiency Protein C deficiency Antithrombin (AT) deficiency Rare disorders Dysfibrinogenemia

58 RISK FACTORS FOR VENOUS THROMBOSIS
Acquired disorders Malignancy Surgery, especially orthopedic Trauma Pregnancy Oral contraceptives Immobilization Antiphospholipid antibody syndrome Myeloproliferative disorders Polycythemia vera Essential thrombocythemia Presence of a central venous catheter Congestive failure Hormone replacement therapy Tamoxifen, Thalidomide, Lenalidomide PNH IBD Nephrotic syndrome Behcet disease

59 SCREENING FOR HYPERCOAGULABLE STATE
Screening for a hypercoagulable state is not generally recommended unless the results are likely to change subsequent therapy for the patient or family members There is currently no consensus regarding who to test for inherited thrombophilia

60 SCREENING FOR HYPERCOAGULABLE STATE
Only patients with one or more of the following: Initial thrombosis occurring prior to age 50 without an immediately identified risk factor (ie, idiopathic or unprovoked venous thrombosis) A family history of venous thromboembolism (ie, first-degree relatives with VTE prior to age 50) Recurrent venous thrombosis Thrombosis occurring in unusual vascular beds such as portal, hepatic, mesenteric, or cerebral veins A history of warfarin-induced skin necrosis, which suggests protein C deficiency

61 THROMBOPHILIA WORK-UP
Antithrombin Protein C Protein S Factor VIII level Factor V Leiden Antiphospholipid antibodies Lupus anticoagulant Prothrombin gene mutation

62 Thrombophilia workup:Effects of anticoagulant therapy and acute thrombosis
Confounding Factors Hypercoagulable disorder for testing Coumadin therapy Heparin therapy Acute thrombosis NC; Rarely increased Lowered Can be lowered Antithrombin (deficiency) NC Antiphospholipid antibodies Factor V Leiden Acute phase reactant. Do not test while inflammation is still present. Factor VIII level False positives possible Cannot measure Lupus anticoagulant Cannot measure• Can be lowered* Protein C (deficiency) Protein S (deficiency) Prothrombin gene mutation

63 BEHCET’S DISEASE

64 DEFINITION Behcet's disease is a multisystem autoimmune disorder presenting with recurrent oral and genital ulcerations as well as ocular involvement.

65 EPIDEMIOLOGY Affects young males and females
Males and females are affected equally Males often have more severe disease Mediterranean region, the Middle East, and the Far East Prevalence ranges from 13.5 to 20 per 100,000 Prevalence in the United States and Europe have ranged from 0.12 to 7.5 per 100,000

66 PATHOGENESIS The etiology and pathogenesis of this syndrome remain obscure Increase of circulating autoantibodies Anti-Enolase of endothelial cells Anti-Selenium binding protein Anti-Saccharomyces cerevisiae antibodies Association of Behcet's disease with HLA-B*5, HLA-B*51 and the MHC Class I region is confirmed An association with ILI0 and the IL23R-ILI2RB2 locus were also observed Perhaps infectious acts as a immune activity trigger

67 PATHOLOGY The classic Behçet’s lesion is
Necrotizing leukocytoclastic obliterative perivasculitis Venous thrombosis Lymphocytic infiltration of capillaries, veins and arteries of all sizes Cellular infiltration is often neutrophils and CD4+ T lymphocytes In some patients, diffuse inflammatory disease, involving all layers of large vessels and resulting to formation of pseudoaneurysms, suggests vasculitis of vasa vasorum.

68 CLINICAL FEATURES Mucocutaneous The recurrent aphthous ulcerations
Are a sine qua non for the diagnosis The ulcers are usually painful Are shallow or deep with a central yellowish necrotic base Appear singly or in crops Are located anywhere in the oral cavity Less than 10 mm in diameter are seen in 85% of patients, while large or herpetiform lesions are less frequent The ulcers persist for 1-2 weeks and subside without leaving scars

69 CLINICAL FEATURES The genital ulcers Pseudofolliculitis
Are less common but more specific Painful Do not affect the glans penis or urethra Produce scrotal scars Pseudofolliculitis Erythema nodosum Acne-like exanthem

70 CLINICAL FEATURES Eye involvement Occurring in 50% of patients
Is usually present at the onset but may also develop within the first few years Includes Anterior uveitis (Iritis ) Posterior uveitis Bilateral pan uveitis with scarring Is the most dreaded complication, since it occasionally progresses rapidly to blindness. Retinal vessel occlusions Optic neuritis

71 CLINICAL FEATURES Articular involvement Seen in a 50% of patients
Non-deforming arthritis or arthralgia Affects knees and ankles

72 CLINICAL FEATURES Vascular involvement Venous involvement
SVT or DVT is seen in 30% of patients The superior vena cava is obstructed occasionally Pulmonary emboli are a rare complication Arterial involvement Occurs in less than 5% of patients Presents with Aortitis Peripheral arterial aneurysm Arterial thrombosis Pulmonary artery vasculitis presenting with dyspnea, cough, chest pain, hemoptysis, and infiltrates on chest roentgenograms has been reported in 5% of patients

73 Behcet’s disease involve blood vessels of all sizes - small, medium, and large - both arteries and veins

74 CLINICAL FEATURES Neurologic involvement 5-10%
Mainly in the parenchymal form (80%) it is associated with brain stem involvement IL-6 is persistently raised in CSF of these patients Dural sinus thrombi (20%) are associated with headache and increased ICP

75 CLINICAL FEATURES Gastrointestinal involvement
Is seen more frequently in patients from Japan Consists of mucosal ulcerations of the gut, resembling Crohn's disease Genital tract involvement Epididymitis is seen in 5% of patients

76 PATHERGY TEST Nonspecific skin inflammatory reactivity to any scratches or intradermal saline injection is a common and specific manifestation A papule or pustul 2 mm or more in size developing 24 to 48 hours after oblique insertion of a 20 to 25 gauge needle 5mm intra-demal, generally performed on the forearm

77 DIFFERENTIAL DIAGNOSIS
Differential diagnosis of recurrent oral ulcers includes Herpes simplex Benign aphthous ulcers Inflammatory bowel disease Stevens-Johnson syndrome SLE Dental prosthetics Oral hygiene products Medications such as methotrexate can cause oral ulcers Pemphigoid, pemphigus vulgaris, cicatricial pemphigoid, Lichen planus Linear IgA disease

78 DIFFERENTIAL DIAGNOSIS
Differential diagnosis of genital ulcers include: HSV Syphilis Chancroid Lymphogranuloma venereum Fixed drug reactions Neoplasms Trauma

79 DIFFERENTIAL DIAGNOSIS
Other causes of inflammatory eye disease, neurologic disease, vascular disease, arthritis, are included SLE IBD Sarcoidosis Reactive arthritis Psoriatic arthritis Ankylosing spondylitis Juvenile idiopathic arthritis FMF MS Tuberculosis HIV Malignancies

80 DIAGNOSIS Diagnosis is clinical and based on internationally agreed diagnostic criteria Recurrent oral ulceration plus two of the following: Recurrent genital ulceration Eye lesions Skin lesions Pathergy test

81 LABORATORY FINDINGS Laboratory findings are mainly nonspecific indices of inflammation, such as Leukocytosis Elevated erythrocyte sedimentation rate Elevated C-reactive protein levels

82 TREATMENT Mucocutaneouce involvement
Topical glucocorticoids (triamcinolone) in the form of mouthwash or paste Topical Sucralfate 1g/5mL four times daily as a mouthwash Colchicine In more serious cases, Thalidomide (l00 mg/d) Prednisone starting dose is 15 mg/day, with tapering to 10 mg/day after one week and discontinuation of prednisone entirely over two to three weeks period

83 TREATMENT Uveitis Sight-threatening uveitis
Prednisone(I mg/kg per day) and azathioprine (2-3 mg/kg per day) Sight-threatening uveitis Cyclosporin (5mg/kg) +/- azathioprine Panuveitis refractory or intolerant to other immunosuppressives Anti-TNF therapy

84 TREATMENT Arthritis Colchicine 1 to 2 mg/day, administered in divided doses NSAIDs Prednisone 10 mg/day is an appropriate starting dose Joint complaints not controlled by colchicine

85 TREATMENT CNS-Behcet's syndrome
Prednisone(I mg/kg per day) and azathioprine (2-3 mg/kg per day)

86 PROGNOSIS Behçet’s disease typically has a waxing and waning course characterized by exacerbations and remissions The disease appears to be more severe in young, male, and Middle Eastern or Far Eastern patients The severity of the syndrome usually abates with time Apart from the patients with CNS-Behcet's syndrome and major vessel disease, the life expectancy seems to be normal and the only serious complication is blindness

87 Oral aphthous on tongue

88 Oral aphthous

89 Dilated superficial veins

90 Erythema nodosum

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92 Oral aphthous


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