Behcets Syndrome is a multisystem disorder characterized by recurrent oral and genital ulcers. It predominantly affects young males and females and can cause ocular involvement leading to blindness. Pathophysiology involves systemic perivasculitis with endothelial inflammation. Clinical features include ulcers, skin lesions, eye involvement, arthritis, and thrombosis. Treatment involves topical steroids for ulcers, thalidomide for severe cases, aspirin for thrombosis, and systemic steroids and immunosuppressants like azathioprine for ocular and neurological involvement.

1. Behcets Syndrome
 Multisystem disorder
 Recurrent oral genital ulcerations
 Ocular involvement
 Young males and females
 Males – more severe disease

2. Pathophysiology
 Systemic perivasculitis with early neutrophil
infiltration and endothelial swelling
 Increased numbers of Th1, Th17, CD8+ and γδ
T cells
 HLA B 51 association

3. Clinical features
 Recurrent aphthous ulcerations
 Usually painful, shallow or deep with central
yellowish necrotic base, single or in crops,
anywhere in oral cavity
 Small ulcers in 85% pts
 Persist for 1-2 weeks, heal w/o scar
 Genital ulcers – less common , more specific
 Painful , don’t affect glans / urethra – scrotal
scars +

4.  Skin involvement – 80% pts
 Folliculitis
 Erythema nodosum
 Sweet syndrome
 Pyoderma gangrenosum
 Pathergy test +ve ( inflammatory skin rxn to
scratches / intradermal saline injn )

5.  Eye involvement
 Scarring and b/l pan-uveitis – most dreaded
complication
 Progress rapidly to blindness
 50% pts
 Usually present at onset, may develop within 1st
few years
 Optic neuritis, retinal vessel occlusion maybe
seen

6.  Non deforming arthritis / arthralgia in 50% pts
 Affects knee and ankles
 Superficial / deep vein thrombosis – 30% of pts
 Pulm emboli – rare complication
 Arterial involvement – less than 5% pts ,
presents with aortitis/ peripheral A aneurysm ,
arterial thrombosis

7.  Pulmonary A vasculitis – 5% patients
 Neurological involvement – 5-10%
 Serious prognosis
 LABS – Raised ESR, CRP

9. Treatment
 Mucus membrane involvement – responds to
topical glucocorticoids
 Thalidomide (100mg/d) – effective in serious
cases
 Thrombophlebitis – 325 mg/d Aspirin
 Uveitis , CNS involvement – systemic
glucocorticoid therapy ( prednisolone
1mg/kg/d) and Azathioprine (2-3mg/kg/d)

11. Common features
 Present as progressive, symmetric muscle
weakness
 Increasing difficulty performing everyday tasks
 Fine movements – affected late
 Ocular muscles – spared
 Facial muscles spared
 Pharyngeal , neck muscles – often involved - >
dysphagia, head drop

12. Polymyositis
 Mimics other myopathies
 Detected late
 Subacute inflammatory myopathy
 Occurs in association with systemic
autoimmune/CTD/Viral/bacterial infn

13. Clinical features
 Typical presentation of polymyositis -
symmetrical proximal muscle weakness, usually
lower limbs > upper limbs.
 Onset - 40 and 60 years of age ,typically gradual,
over a few weeks.
 Myositis - usually widespread but focal disease
can also occur (e.g. orbital myositis).
 Affected patients report difficulty rising from a
chair, climbing stairs and lifting, sometimes in
combination with muscle pain.

14.  Systemic features of fever, weight loss and
fatigue are common.
 Respiratory or pharyngeal muscle involvement
 ventilatory failure / aspiration - requires
urgent treatment.
 Interstitial lung disease occurs in up to 30% of
patients and is strongly associated with the
presence of antisynthetase (Jo-1) antibodies.

15.  Dermatomyositis presents
similarly but in combination
with characteristic skin lesions.
 These include Gottron's
papules, which are scaly
erythematous or violaceous
psoriaform plaques occurring
over the extensor surfaces of
proximal and distal IPJs,

16.  Heliotrope rash which is a
violaceous discoloration of the
eyelid in combination with
periorbital oedema.
 Similar rashes occur on the
upper back, chest and shoulders
('shawl' distribution).
 Periungual nail-fold capillaries
are often enlarged and tortuous.
 There is about a threefold
increased risk of malignancy in
patients with dermatomyositis
and polymyositis.

17. Extramuscular manifestations
 Systemic symptoms – fever, malaise, wt loss,
arthralgia, Raynauds.
 Joint contractures
 Dysphagia , GI symptoms
 Cardiac disturbances – AV conduction defects,
CCF, tachyarrhythmias, DCM
 Pulmonary dysfunction – due to thoracic muscle
weakness

18.  Subcutaneous calcifications – in
dermatomyositis
 Arthralgias, synovitis, deforming arthropathy
with subluxation in the interphalangeal joints

20. Differential diagnosis
 Subacute or chronic progressive muscle
weakness – Spinal muscular atrophy / ALS ->
EMG may aid in diagnosis
 Acute muscle weakness – GBM, transverse
myelitis, poliomyelitis
 Myofascitis
 Necrotising Autoimmune myositis
 Drug induced myopathies

21. Investigations
 Muscle biopsy is a pivotal investigation
and shows the typical features of fibre
necrosis, regeneration and
inflammatory cell infiltrate.
 EMG can confirm the presence of
myopathy and exclude neuropathy.
 MRI will identify areas of abnormal
muscle whenever, a biopsy may be
normal, particularly if myositis is
patchy.
 Serum levels of CK are usually raised
and are a useful measure of disease
activity.
 Screening for underlying malignancy
should be undertaken routinely.
Muscle biopsy from a patient
with dermatomyositis
demonstrates atrophy of the
fibers at the periphery of the
fascicle (perifascicular atrophy)

23. Management
 Oral corticosteroids (e.g. prednisolone 1mg/kg/day)
are the mainstay of initial treatment but high-dose
intravenous methylprednisolone (1 g/day for 3 days)
may be required in patients with respiratory or
pharyngeal weakness.
 If there is a good response, steroids should be reduced
by approximately 5 to 10 mg every 3 – 4 weeks till
lowest possible dose is reached.
 Although most patients have an initial response to
steroids, most need additional immunosuppressive
therapy.

24.  Azathioprine – well tolerated, effective as long term
therapy.
 Dose – 3mg/kg/day
 Methotrexate – faster onset of action
 Dose – 7.5 mg weekly for 1st 3 weeks, gradual
escalation by 2.5 mg per week  target dose of 25 mg
weekly
 MMF – faster action than AZT – 2.5 – 3 g/day in 2
divided doses
 Cyclosporine – mild benefit
 Cyclophosphamide – 0.5 – 1 g/m2 – limited success

25.  Intravenous immunoglobulin may be effective in
refractory cases. 2g/d over 2 – 5 days.
 Benefit – short lived and repeat infusions every
6 – 8 weeks maybe needed.

26. Prognosis
 5 yr survival – 95% if treated
 10 yr survival – 84%
 Poor prognosis – severely symptomatic at
presentation, delayed treatment
 Older patients – poor prognosis