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

Secondary OA

Carpal tunnel syndromeProximal muscle weakness with normal CK

• Raynaud's Phenomenon

Acromegaly

Chondrocalcinosis

Acromegaly: hand (radiograph)

Cushing's Syndrome

Proximal muscle weakness

Osteonecrosis

OsteoporosisSteroid withdrawal syndrome

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 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

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– 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

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