Systemic Diseases and Musculoskeletal System
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Transcript of Systemic Diseases and Musculoskeletal System
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Systemic Diseases and Musculoskeletal System
Johan van Rensburg
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Systemic Diseases and Musculoskeletal System
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Endokrine associated
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When to suspect occult endocrinopathy?
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Entrapment Neuropathy (Carpal tunnel Syndrome)
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Calcium Pyrophosphate Dihydrate (CPPD) arthropathy
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Diffuse myalgia (with or without muscle weakness)
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Raynaud's phenomenon
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Diabetes Mellitus
• Intrinsic complications• Conditions with increased incidence
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Intrinsic complications
Neuro-vascular prominent
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Diabetic cheiroathropathy (diabetic hand syndrome of limited joint mobility)
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Diabetic arthropathy: neuropathic joint disease, feet (clinical and radiograph)
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Diabetic osteolysis
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Diabetic amyotrophy
Diabetic neuropathy characterized by painful muscle wasting and weakness. It affects the lower limbs and is typically asymmetric.
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Conditions with increased incidence
• Increased deposition of abnormal protein• Increase in growth factors• Decreased immunity
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Frozen shoulder (periarthritis of the shoulder)
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Reflex sympathetic dystrophy (shoulder hand syndrome)
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Flexor tenosynovitis of the hand
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Dupuytren's contracture
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Carpal tunnel syndrome
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Diffuse idiopathic skeletal hyperostosis (DISH)
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Septic joint/osteomielitis
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Thyroid
• Hypothyroidism• Hyperthyroidism
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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
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Hyperthyroidism
• Thyroid acropachy (1%)• Painless proximal muscle weakness • Osteoporosis• Adhesive capsulitis of the shoulder
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Hyperthyroidism: acropachy, hand (clinical and radiograph)
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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
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Hypoparathyroidism
• Hypocalcaemia
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Acromegaly
Secondary OA
Carpal tunnel syndromeProximal muscle weakness with normal CK
• Raynaud's Phenomenon
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Acromegaly
Chondrocalcinosis
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Acromegaly: hand (radiograph)
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Cushing's Syndrome
Proximal muscle weakness
Osteonecrosis
OsteoporosisSteroid withdrawal syndrome
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Hematological disorders
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Bone pain (worse at night)
• Hypercoagulabilty– Antiphosfolipid syndrome
• Sickle cell disease• Leukemia• Mieloma
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Sickle-cell disease: bone infarcts, upper tibiae (radiograph)
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Hemophilia: knees (radiograph)
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Other Malignancies
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Metastatic adernocarcinoma: hand (radiograph)
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Osteosarcoma: femur (radiograph)
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Other
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Infections
• TB– Osteomielitis– Spinal involvement
• Brucella– Sero-negative arthritis– Involvement of the spine
• Virus– Myopathy
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Syphilitic neuropathic joint disease: knees
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Hemochromatosis: hands (radiograph)
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Electrolyte disorders
• Myopathies– Low Potassium– Low magnesium– Low/high calcium
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Amyloidosis
• Associated with– Chronic dialysis– Mieloproliferative diseases– Chronic inflammation
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Amyloidosis: shoulder (clinical and photomicrographs)
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Amyloidosis: tongue
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Granulomas, abnormal protein and chronic inflammation
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Erythema nodosum: legs
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Sarcoid arthritis: hands
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Systemic Vasculitis
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Systemic Vasculitis
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Classification
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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
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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
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Specific features
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Organ involvement in vasculitis
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Large-size vessel
• Giant cell (temporal) arteritis• Takayasus arteritis
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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
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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
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Takayasus arteritis
• Granulomatous• Aorta and major branches• Clinical Features• Special investigations
– Arteriogram• Narrowing/Occlusion
– aorta and main braches
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Takayasus arteritisClinical Features
• Usually patients <50 years• Extremities
– Claudicating• Especially upper
– Decrease in brachial artery pulse– Blood pressure difference
• >10mmHG• Between arms
• Bruit– Subclavian arteries– Aorta
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Medium-sized vessel
• Polarteritis nodosa (Classic poliarteritis nodosa)• Kawasaki disease
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Polarteritis nodosa (Classic poliarteritis nodosa)
• Necrotizing inflammation• Association
– Hepatitis B infection• Clinical• Special investigations
– P-ANCA (30%)• Mieloperoksidase
– Arteriogram• Microaneurisms
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Polarteritis nodosa (Classic poliarteritis nodosa)Clinical
• Without– Glomerulonephritis– Vasculitis in arterioles, capilaries, venules
• Peripheral nervous• Symptoms of systemic inflammation
– Malaise– Myalgia– Fatigue– Loss of weight– Fever
• Musculoskeletal• Skin• Kidney
– Larger vessels– Hypertension– Microaneurisms
• GIT• Rare
– Central nervous– Cardiac – Lungs– Eyes
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Kawasaki disease
• Coronary arteries• May involve
– Aorta and Veins• Usually Children• Association
– Mucocutaneous lymph node syndrome
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Small-sized vessel
• Wegener's granulomatosis• Churg-Strauss syndrome• Microscopic polyangiitis (Microscopic polyarteritis)• Henoch-Shonlein purpura• Essential Cryoglobulinemia vascultis• Cutaneous leukocytoclatic angiitis
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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
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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
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Microscopic polyangiitis (Microscopic polyarteritis)
• Necrotizing– Few/No
• Immune deposits• Capillaries, Venules, arterioles• Kidney
– Necrotizing Glomerulonepritis• Lung
– Pulmonary capillaritis
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Henoch-Shonlein purpura
• Immune deposits– IgA
• Organs– Skin
• Palpable purpura– Gut
• Bowel angina– Worse after meals
• Bloody diarrhea– Kidney
• Glomeruli– Musculoskeletal
• Arthralgias/arthritis
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Essential Cryoglobulinemia vascultis
• Immune deposits• Organs
– Skin• Cold extremities
– Ulcers• Raynaud's
– Kidney• Glomeruli
– Peripheral neuropathy– Artthralgia– Fatigue
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Cutaneous leukocytoclatic angiitis
• Isolated coetaneous• Without
– Systemic vasculitis– Glomerulonephritis
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Important clinical features
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Emergencies
• Central nervous system• Mononeuritis• Mononeuritis multiplex• Kidney• Lung• Eye
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Treatment
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Medical
• Treat underlying cause– Malignancy– Connective tissue disease– Drug reaction– Infections
• Immunosuppressant– Corticosteroids– Cyclophosphamide– Azathioprine– Mofetil– Cyclosporine
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Surgical
• Takayasu• PAN
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