Download Vasculitis and Systemic Diseases and MS system

Systemic Diseases and
Musculoskeletal System
Johan van Rensburg
Systemic Diseases and
Musculoskeletal System
Endokrine associated
When to suspect occult
endocrinopathy?
Entrapment Neuropathy
(Carpal tunnel Syndrome)
Calcium Pyrophosphate Dihydrate (CPPD)
arthropathy
Diffuse myalgia
(with or without muscle weakness)
Raynaud's phenomenon
Diabetes Mellitus
• Intrinsic complications
• Conditions with increased incidence
Intrinsic complications
Neuro-vascular prominent
Diabetic cheiroathropathy (diabetic hand
syndrome of limited joint mobility)
Diabetic arthropathy: neuropathic joint disease,
feet (clinical and radiograph)
Diabetic osteolysis
Diabetic amyotrophy
Diabetic neuropathy characterized by painful
muscle wasting and weakness.
It affects the lower limbs and is typically asymmetric.
Conditions with increased
incidence
• Increased deposition of abnormal protein
• Increase in growth factors
• Decreased immunity
Frozen shoulder (periarthritis of the shoulder)
Reflex sympathetic dystrophy (shoulder hand
syndrome)
Flexor tenosynovitis of the hand
Dupuytren's contracture
Carpal tunnel syndrome
Diffuse idiopathic skeletal hyperostosis
(DISH)
Septic joint/osteomielitis
Thyroid
• Hypothyroidism
• Hyperthyroidism
Hypothyroidism
• Tunnel (carpal) syndrome
• Raynaud's phenomenon
• Aching muscle with findings indistinguishable of
fibromyalgia
• Proximal muscle weakness and stiffness with
elevated CK
• Myxedematous arthropathy in severe
hypothyroidism
• Auto-immune thyroiditis and other auto-immune
diseases
Hyperthyroidism
•
•
•
•
Thyroid acropachy (1%)
Painless proximal muscle weakness
Osteoporosis
Adhesive capsulitis of the shoulder
Hyperthyroidism: acropachy, hand (clinical and
radiograph)
Hyperparathyroidism
• Painless proximal muscle weakness
– (Normal CK BUT Myopathic or neuropathic EMG)
• Chondrocalcinosis with pseudogout attacks
(usually due to CPPD)
• Osteogenic synovitis due to subchondral bony
collapse from thinning of bone (leading to
secondary OA)
• Osteoporosis
• Ectopic soft-tissue calcification
Hypoparathyroidism
• Hypocalcaemia
Acromegaly
•Raynaud's Phenomenon
Secondary OA
Proximal muscle
weakness
with normal CK
Carpal tunnel syndrome
Acromegaly
Chondrocalcinosis
Acromegaly: hand (radiograph)
Cushing's Syndrome
Osteoporosis
Steroid withdrawal syndrome
Osteonecrosis
Proximal muscle weakness
Hematological disorders
Bone pain (worse at night)
• Hypercoagulabilty
– Antiphosfolipid syndrome
• Sickle cell disease
• Leukemia
• Mieloma
Sickle-cell disease: bone infarcts, upper
tibiae (radiograph)
Hemophilia: knees (radiograph)
Other Malignancies
Metastatic adernocarcinoma: hand
(radiograph)
Osteosarcoma: femur (radiograph)
Other
Infections
• TB
– Osteomielitis
– Spinal involvement
• Brucella
– Sero-negative arthritis
– Involvement of the spine
• Virus
– Myopathy
Syphilitic neuropathic joint disease: knees
Hemochromatosis: hands (radiograph)
Electrolyte disorders
• Myopathies
– Low Potassium
– Low magnesium
– Low/high calcium
Amyloidosis
• Associated with
– Chronic dialysis
– Mieloproliferative diseases
– Chronic inflammation
Amyloidosis: shoulder (clinical and
photomicrographs)
Amyloidosis: tongue
Granulomas, abnormal protein and
chronic inflammation
Erythema nodosum: legs
Sarcoid arthritis: hands
Systemic Vasculitis
Systemic Vasculitis
Classification
Chapel Hill Classification
• Large-size vessel
– Giant cell (temporal) arteritis
– Takayasus arteritis
• Medium-sized vessel
– Polarteritis nodosa (Classic poliarteritis nodosa)
– Kawasaki disease
• Small-sized vessel
– Wegener's granulomatosis
– Churg-Strauss syndrome
– Microscopic polyangiitis
• (Microscopic polyarteritis)
– Henoch-Shonlein purpura
– Essential Cryoglobulinemia vascultis
– Cutaneous leukocytoclatic angiitis
Practical Classification
•
Primary vasculitides
– Large, medium and small vessels
•
•
•
Takayasu
Giant cell arteritis
Isolated angiitis of the central nervous
system
– Medium and small vessels
•
•
•
Poliarteritis nodosa
Churg-Strauss syndrome
Wegener's granulomatosis
– Small sized vessels
•
•
•
Microscopic poliangiitis
Henoch-Shonlein purpura
Cutaneous leukocytoclastic angiitis
– Miscellaneous conditions
• Buerger's disease
• Cogan's syndrome
• Kawasaki's disease
• Secondary vasculitides
–
–
–
–
–
Infections
Connective tissue diseases
Malignancy
Drug Hypersensitivity
Mixed essential
crioglobulinemia
– Hipocomplementemic
urticarial
– Post organ transplant
– Pseudovasculitic syndromes
Specific features
Organ involvement in vasculitis
Large-size vessel
• Giant cell (temporal) arteritis
• Takayasus arteritis
Giant cell (temporal) arteritis
• Granulomatous
• Aorta and major branches
• Predilection for Extra cranial braches of carotid artery
– Often temporal artery
• Association
– Polymyalgia rheumatica
• Clinical features
• Special investigations
– Raised ESR
– Anemia
Giant cell (temporal) arteritis
Clinical features
•
•
Usually patients >50 years
Temporal artery
–
–
–
–
–
Thickened
Tender
Decreased pulsation
Necrosis of skin
Eye symptoms
• Blindness
• Emergency
– Jaw claudication
– Headache
– Dizziness
•
Symptoms of systemic inflammation
–
–
–
–
–
Malaise
Myalgia
Fatigue
Loss of weight
Fever
Takayasus arteritis
•
•
•
•
Granulomatous
Aorta and major branches
Clinical Features
Special investigations
– Arteriogram
• Narrowing/Occlusion
– aorta and main braches
Takayasus arteritis
Clinical Features
• Usually patients <50 years
• Extremities
– Claudicating
• Especially upper
– Decrease in brachial artery pulse
– Blood pressure difference
• >10mmHG
• Between arms
• Bruit
– Subclavian arteries
– Aorta
Medium-sized vessel
• Polarteritis nodosa (Classic poliarteritis nodosa)
• Kawasaki disease
Polarteritis nodosa (Classic
poliarteritis nodosa)
• Necrotizing inflammation
• Association
– Hepatitis B infection
• Clinical
• Special investigations
– P-ANCA (30%)
• Mieloperoksidase
– Arteriogram
• Microaneurisms
Polarteritis nodosa (Classic poliarteritis nodosa)
Clinical
•
Without
–
–
•
•
Peripheral nervous
Symptoms of systemic inflammation
–
–
–
–
–
•
•
•
Malaise
Myalgia
Fatigue
Loss of weight
Fever
Musculoskeletal
Skin
Kidney
–
–
–
•
•
Glomerulonephritis
Vasculitis in arterioles, capilaries, venules
Larger vessels
Hypertension
Microaneurisms
GIT
Rare
–
–
–
–
Central nervous
Cardiac
Lungs
Eyes
Kawasaki disease
• Coronary arteries
• May involve
– Aorta and Veins
• Usually Children
• Association
– Mucocutaneous lymph node syndrome
Small-sized vessel
•
•
•
•
•
•
Wegener's granulomatosis
Churg-Strauss syndrome
Microscopic polyangiitis (Microscopic polyarteritis)
Henoch-Shonlein purpura
Essential Cryoglobulinemia vascultis
Cutaneous leukocytoclatic angiitis
Wegener's granulomatosis
• Granulomatous
• Necrotizing glomerulonephritis
• Clinical
– <50 years
– Respiratory tract
• Saddle nose
• Perforation of nose septum
• Lung infiltrates
– Interstitial
– Nodules
• Cavities in lung
– Eye
• Uviitis
• Pseudo tumor
– Kidney
• Glomerulonephritis
– Arthritis
– Skin
– Neurologic
• Special investigations
– c-ANCA
• Proteinase 3
– X-rays
Churg-Strauss syndrome
•
Granulomatous
– Eosinophil-rich
•
Associations
– Asthma
– Eosinophilia
•
Clinical
– Respiratory tract
• Asthma
• Pulmonary infiltrates
– Migratory
• Para nasal sinus abnormality
– Neuropathy
• Mono/Poly
–
–
–
–
Musculoskeletal
Skin
GIT
Kidney
•
Special investigations
–
Biopsy
Microscopic polyangiitis
(Microscopic polyarteritis)
• Necrotizing
– Few/No
• Immune deposits
• Capillaries, Venules, arterioles
• Kidney
– Necrotizing Glomerulonepritis
• Lung
– Pulmonary capillaritis
Henoch-Shonlein purpura
• Immune deposits
– IgA
• Organs
– Skin
• Palpable purpura
– Gut
• Bowel angina
– Worse after meals
• Bloody diarrhea
– Kidney
• Glomeruli
– Musculoskeletal
• Arthralgias/arthritis
Essential Cryoglobulinemia
vascultis
• Immune deposits
• Organs
– Skin
• Cold extremities
– Ulcers
• Raynaud's
– Kidney
• Glomeruli
– Peripheral neuropathy
– Artthralgia
– Fatigue
Cutaneous leukocytoclatic angiitis
• Isolated coetaneous
• Without
– Systemic vasculitis
– Glomerulonephritis
Important clinical features
Emergencies
•
•
•
•
•
•
Central nervous system
Mononeuritis
Mononeuritis multiplex
Kidney
Lung
Eye
Treatment
Medical
• Treat underlying cause
–
–
–
–
Malignancy
Connective tissue disease
Drug reaction
Infections
• Immunosuppressant
–
–
–
–
–
Corticosteroids
Cyclophosphamide
Azathioprine
Mofetil
Cyclosporine
Surgical
• Takayasu
• PAN
END