SYSTEMIC INFLAMMATORY VASCULITIS - Clinical features, Investigations

Vasculitis is inflammation of the blood vessel walls and can be seen in many diseases including SLE, RA, polymyositis and some allergic drug reactions. The term ‘systemic vasculitides' describes a group of multisystem disorders in which vasculitis is the principal feature and classification is based on the size of the vessels affected (Table 7.11). They are all associated with anaemia and a raised ESR and all are rare except for giant cell (temporal) arteritis.

Table 7.11 Classification of systemic vasculitis
Large (aorta and its major branches)
Giant-cell arteritis/polymyalgia rheumatica
Takayasu’s arteritis
Medium (main visceral vessels, e.g. renal, coronary)
Polyarteritis nodosa
Kawasaki’s disease (affects children <5 years)
Small (small arteries, arterioles, venules and capillaries)
ANCA positive
Microscopic polyangiitis
Wegener’s granulomatosis
Churg–Strauss syndrome
ANCA negative
Henoch–Schönlein purpura
Cutaneous leucocytoclastic vasculitis
Essential cryoglobulinaemia
ANCA, antineutrophil cytoplasmic antibodies (p. 299).

Polymyalgia and giant cell arteritis

Polymyalgia (PMR) and giant cell arteritis (GCA) are systemic illnesses affect-ing patients older than 50 years. Both are associated with the finding of GCA on temporal artery biopsy. Patients may have symptoms and signs limited to PMR or to GCA throughout the course of their illness while others have manifestations of both.

Clinical features

PMR causes an abrupt onset of stiffness and intense pain in the muscles of the neck and shoulder, and hips and lumbar spine. Significant objective weakness is uncommon. There may be constitutional symptoms, with malaise, fever, weight loss and anorexia. Arteritic involvement by inflamma-tion is most frequently noticed in the superficial temporal arteries and causes headache, tenderness over the scalp or temple (combing the hair may be painful), and claudication of the jaw when eating. GCA affecting the verte-brobasilar, and sometimes the carotid, circulation may result in stroke. The most devastating complication of GCA is sudden loss of vision (which may be permanent) due to involvement of the ophthalmic artery. Early recognition and treatment is therefore essential.

Investigations

Treatment is started based on a clinical diagnosis and investigations showing a very high ESR (around 100 mm/h) and CRP. There is often a normochromic, normocytic anaemia. Temporal artery biopsy is performed if GCA is sus-pected and should be performed before or within a week of starting corticosteroids.

Management

Treatment is with corticosteroids, prednisolone 10-15 mg/day for PMR and 60 mg/day for GCA. The dose is gradually reduced by weekly decrements of

5 mg. Once 10 mg is reached a reduction of 1 mg every 2-4 weeks is usually sufficient. The dose is titrated against symptoms and the ESR. Prophylaxis against steroid-induced osteoporosis should be given (p. 314). The disease may relapse when steroid treatment is stopped.

Takayasu’s arteritis

This is rare except in Japan. Vasculitis involving the aortic arch and other major arteries causes hypertension, absent peripheral pulses, strokes and cardiac failure. Treatment is with corticosteroids.

Polyarteritis nodosa

Polyarteritis nodosa (PAN) predominantly affects middle-aged men. Its asso-ciation with hepatitis B antigenaemia in some cases suggests a vasculitis secondary to the deposition of immune complexes. There is a necrotizing arteritis associated with microaneurysm formation, thrombosis and infarc-tion. Clinical features include fever, malaise, weight loss, mononeuritis mul-tiplex, abdominal pain (resulting from visceral infarcts), renal impairment and hypertension. The lungs are rarely involved (cf. antineutrophilic cytoplasmic antibodies (ANCA)-positive vasculitides). The diagnosis is made on angio-graphy (microaneurysms in hepatic, intestinal or renal vessels) or biopsy of an affected organ, often the kidney, which shows features listed above. Treatment is with corticosteroids unusually in combination with immuno-suppressive drugs (azathioprine, cyclophosphamide).

Microscopic polyarteritis (polyangiitis)

This involves the lungs and the kidney where it results in haemoptysis, haematuria, proteinuria and Progressive renal failure. Other features include arthralgia and purpuric rashes. Diagnosis is by renal biopsy and measure-ment of serum perinuclear (p)-ANCA, present in 60%; see p. 299). Treatment is similar to PAN.

Churg-Strauss syndrome

This is rare and characterized by a triad of asthma, eosinophilia and a systemic vasculitis affecting the peripheral nerves and skin (nodules, petechiae, purpura) but kidney involvement is uncommon. Treatment is similar to PAN.

Henoch-Schỏnlein purpura

This condition is most commonly seen in children and presents as a purpuric rash, mainly on the legs and buttocks. Abdominal pain, arthritis, haematuria and nephritis also occur. It is characterized by vascular deposition of Iga-dominant immune complexes, and the onset is often preceded by an acute upper respiratory tract infection. Recovery is usually spontaneous.

Cryoglobulinaemic vasculitis

Cryoglobulins are immunoglobulins and complement components that pre-cipitate reversibly in the cold. Types include essential cryoglobulinaemia (no underlying disease) or associated with infection (e.g. hepatitis B and C, HIV) or autoimmune disease. There is involvement of the skin (purpura, arthralgia, leg ulcers), kidneys (glomerulonephritis) and polyneuropathy. Treatment is similar to PAN.

Behçet’s disease

This is a rare multisystem chronic disease of unknown cause and most common in Turkey, Iran and Japan. It is characterized by recurrent oral ulceration. Diagnosis is clinical and requires the presence of oral ulceration and any two of the following: genital ulcers, eye lesions (uveitis, retinal vascular lesions), skin lesions (erythema nodosum, papulopustular lesions), or a positive skin pathergy test (skin injury, e.g. needle prick, leads to pustule formation within 48 hours). Other features include arthritis, gastrointestinal ulceration with pain and diarrhoea, pulmonary and renal lesions, meningo-encephalitis, and organic confusional states. Treatment is with immuno-suppressive therapy (steroids, azathioprine, ciclosporin) or occasionally thalidomide.

Ebook Essentials of Kumar and Clark's Clinical Medicine, 5e

1. Ethics and communication

Ethics and communication

2. Infectious diseases

Infectious diseases

3. Gastroenterology and nutrition

Gastroenterology and nutrition

4. Liver, biliary tract and pancreatic disease

Liver, biliary tract and pancreatic disease
LIVER BIOCHEMISTRY AND LIVER FUNCTION TESTS
SYMPTOMS AND SIGNS OF LIVER DISEASE
JAUNDICE
HEPATITIS
NON - ALCOHOLIC FATTY LIVER DISEASE (NAFLD)
CIRRHOSIS
COMPLICATIONS AND EFFECTS OF CIRRHOSIS
LIVER TRANSPLANTATION
TYPES OF CHRONIC LIVER DISEASE AND CIRRHOSIS
PRIMARY SCLEROSING CHOLANGITIS
BUDD - CHIARI SYNDROME
LIVER ABSCESS
LIVER DISEASE IN PREGNANCY
LIVER TUMOURS
GALLSTONES
THE PANCREAS
CARCINOMA OF THE PANCREAS
NEUROENDOCRINE TUMOURS OF THE PANCREAS

5. Haematological disease

Haematological disease
ANAEMIA
Assessment and treatment of suspected neutropenic sepsis
HAEMOLYTIC ANAEMIA
INHERITED HAEMOLYTIC ANAEMIAS
ACQUIRED HAEMOLYTIC ANAEMIA
MYELOPROLIFERATIVE DISORDERS
THE SPLEEN
BLOOD TRANSFUSION
THE WHITE CELL
HAEMOSTASIS AND THROMBOSIS
THROMBOSIS
THERAPEUTICS

6. Malignant disease

Malignant disease
MYELOABLATIVE THERAPY AND HAEMOPOIETIC STEM CELL TRANSPLANTATION
THE LYMPHOMAS
THE PARAPROTEINAEMIAS
PALLIATIVE MEDICINE AND SYMPTOM CONTROL

7. Rheumatology

Rheumatology
COMMON INVESTIGATIONS IN MUSCULOSKELETAL DISEASE
COMMON REGIONAL MUSCULOSKELETAL PROBLEMS
BACK PAIN
OSTEOARTHRITIS
INFLAMMATORY ARTHRITIS
THE SERONEGATIVE SPONDYLOARTHROPATHIES
Clinical features, Investigations
INFECTION OF JOINTS AND BONES
AUTOIMMUNE RHEUMATIC DISEASES
SYSTEMIC INFLAMMATORY VASCULITIS
DISEASES OF BONE
THERAPEUTICS

8. Water, electrolytes and acid–base balance

WATER AND ELECTROLYTE REQUIREMENTS
BODY FLUID COMPARTMENTS
REGULATION OF BODY FLUID HOMEOSTASIS
PLASMA OSMOLALITY AND DISORDERS OF SODIUM REGULATION
DISORDERS OF POTASSIUM REGULATION
DISORDERS OF MAGNESIUM REGULATION
DISORDERS OF ACID - BASE BALANCE
THERAPEUTICS

9. Renal disease

Renal disease
INVESTIGATION OF RENAL DISEASE
GLOMERULAR DISEASES
NEPHROTIC SYNDROME
URINARY TRACT INFECTION
TUBULOINTERSTITIAL NEPHRITIS
HYPERTENSION AND THE KIDNEY
RENAL CALCULI AND NEPHROCALCINOSIS
URINARY TRACT OBSTRUCTION
ACUTE RENAL FAILURE/ACUTE KIDNEY INJURY
CHRONIC KIDNEY DISEASE
RENAL REPLACEMENT THERAPY
CYSTIC RENAL DISEASE
TUMOURS OF THE KIDNEY AND GENITOURINARY TRACT
DISEASES OF THE PROSTATE GLAND
TESTICULAR TUMOUR
URINARY INCONTINENCE

10. Cardiovascular disease

COMMON PRESENTING SYMPTOMS OF HEART DISEASE
INVESTIGATIONS IN CARDIAC DISEASE
CARDIAC ARRHYTHMIAS
HEART FAILURE
ISCHAEMIC HEART DISEASE
RHEUMATIC FEVER
VALVULAR HEART DISEASE
PULMONARY HEART DISEASE
MYOCARDIAL DISEASE
CARDIOMYOPATHY
PERICARDIAL DISEASE
SYSTEMIC HYPERTENSION
ARTERIAL AND VENOUS DISEASE
ELECTRICAL CARDIOVERSION
DRUGS FOR ARRHYTHMIAS
DRUGS FOR HEART FAILURE
DRUGS AFFECTING THE RENIN - ANGIOTENSIN SYSTEM
NITRATES, CALCIUM - CHANNEL BLOCKERS AND POTASSIUM - CHANNEL ACTIVATORS

11. Respiratory disease


Respiratory disease
TUBERCULOSISnd
DIFFUSE DISEASES OF THE LUNG PARENCHYMA
OCCUPATIONAL LUNG DISEASE
CARCINOMA OF THE LUNG
DISEASES OF THE CHEST WALL AND PLEURA
DISORDERS OF THE DIAPHRAGM

12. Intensive care medicine

Intensive care medicine

13. Drug therapy, poisoning, and alcohol misuse

Drug therapy, poisoning, and alcohol misuse

14. Endocrine disease

Endocrine disease
PITUITARY HYPERSECRETION SYNDROMES
THE THYROID AXIS
MALE REPRODUCTION AND SEX
FEMALE REPRODUCTION AND SEX
THE GLUCOCORTICOID AXIS
THE THIRST AXIS
DISORDERS OF CALCIUM METABOLISM
DISORDERS OF PHOSPHATE CONCENTRATION
ENDOCRINOLOGY OF BLOOD PRESSURE CONTROL
DISORDERS OF TEMPERATURE REGULATION
THERAPEUTICS

15. Diabetes mellitus and other disorders of metabolism

DIABETES MELLITUS
DIABETIC METABOLIC EMERGENCIES
COMPLICATIONS OF DIABETES
SPECIAL SITUATIONS
HYPOGLYCAEMIA IN THE NON - DIABETIC
DISORDERS OF LIPID METABOLISM
THE PORPHYRIAS

16. The special senses

THE EAR
THE NOSE AND NASAL CAVITY
THE THROAT
THE EYE

17. Neurology

COMMON NEUROLOGICAL SYMPTOMS
COORDINATION OF MOVEMENT
THE CRANIAL NERVES
COMMON INVESTIGATIONS IN NEUROLOGICAL DISEASE
UNCONSCIOUSNESS AND COMA
STROKE AND CEREBROVASCULAR DISEASE
EPILEPSY AND LOSS OF CONSCIOUSNESS
NERVOUS SYSTEM INFECTION AND INFLAMMATION
HYDROCEPHALUS
HEADACHE, MIGRAINE AND FACIAL PAIN
SPINAL CORD DISEASE
DEGENERATIVE NEURONAL DISEASES
DISEASES OF THE PERIPHERAL NERVES
MUSCLE DISEASES
MYOTONIAS
DELIRIUM
THERAPEUTICS

18. Dermatology

Dermatology

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