Vasculitis Syndrome

Vasculitis is a disease of blood vessels with in­flammation and damage. Vasculitis may be a disease of the blood vessels itself or it may be secondarily affected due to a systemic disease. destruction of blood vessel walls, with subsequent aneurysm formation, bleeding, thrombosis, or ischemia in the various vascular beds and organs Differential Diagnosis Consists of a heterogeneous group of diseases depending on size, type, and location of the blood vessel involved.

• ›Small-vessel vasculitis:

  • Wegener granulomatosis
  • Viral-associated vasculitis
  • Rheumatic-associated vasculitis
  • Microscopic polyangiitis
  • Hypersensitivity vasculitis
  • Henoch-Schönlein purpura
  • Essential cryoglobulinemic vasculitis
  • Churg-Strauss arteritis

• ›Medium-vessel vasculitis:

  • Isolated CNS vasculitis
  • Kawasaki disease
  • Polyarteritis nodosa

• ›Large-vessel vasculitis:

  • Giant cell arteritis
  • Takayasu arteritis

Vasculitis syndromes:

• Wegener’s granulomatosis Churg Strauss syndrome Polyarteritis nodosa
• Giant cell arteritis Takayasu arteritis
• Henoch Schonlein purpura Behcet’s syndrome
• Cogan syndrome Kawasaki disease.

Secondary causes:

• Infection
• Malignancy
• Rheumatic disease Serum sickness Drug-induced vasculitis.

Mechanism of Vasculitis:

• Immune complex formation
• Pathologic T Iymphocyte responses Antineutrophil cytoplasmic antibodies (ANCA).

Vasculitis Syndrome Pathophysiology —

• Three major immunopathogenic mechanisms have been proposed.
• Pathogenic T-lymphocyte response: Giant cell arteritis and Takayasu arteritis
• Immune-complex formation: SLE, polyarteritis nodosa, and essential mixed cryoglobulinemia
• Antineutrophil cytoplasmic antibodies (ANCAs): Wegener granulomatosis, microscopic polyangiitis, and Churg-Strauss syndrome

WEGENER’S GRANULOMATOSIS

• There is granulomatous vasculitis of small arteries and veins.
• Involves upper and lower respiratory tracts. There is renal involvement in the form of glomeru­lonephritis.
• Eye involvement can occur, like conjunctivitis, scleri­tis, uveitis.
• Skin may show papules, vesicles, purpura and sub­cutaneous nodules.

WEGENER’S GRANULOMATOSIS  Diagnosis

• · There are necrotizing granulomatous lesions in tissue biopsy.
• · Renal biopsy shows immune glomerulo-nephri­tis.
• · Positive anti proteinase 3 ANCA.

WEGENER’S GRANULOMATOSIS  Treatment

• Glucocorticoids
• Cyclophosphamide – 2 mg/kg/day Blood counts must be monitored Prognosis of the disease is good.

CHURG-STRAUSSSYNDROME

• Also called allergic angiitis and granulomatosis.
• There is asthma, eosinophilia and vasculitis of sev­eral organs.
• Involves small and medium-sized blood vessels There is infiltration of tissues with eosinophils.
• Organs involved are lungs, skin, heart, kidney, pe­ripheral nervous system, gastrointestinal tract.

CHURG-STRAUSSSYNDROME Symptoms

• Fever, malaise, anorexia, weight loss. There are severe asthmatic attacks. There is mononeuritis multiplex. There is allergic rhinitis and sinusitis.

CHURG-STRAUSSSYNDROME Lab findings

• · There is severe eosinophilia
• · ANCA is positive.

CHURG-STRAUSSSYNDROME Treatment

• Glucocorticoids
• Cyclophosphamide.

POLYARTERITIS

• NODOSA Also called Classic PAN.
• Involves small and medium-sized muscular ar­teries.
• Involves kidneys.

POLYARTERITIS  Clinical findings

• · Fever, weight loss, malaise, headache, abdomi­nal pain, myalgias
• · Renal involvement leading to hypertension and renal failure
• · Gastrointestinal complications.

Lab findings

• · Leucocyte count is increased
• · Anaemia
• · Hypergammaglobulinemia
• · Tissue biopsy shows inflammation.
• POLYARTERITIS Angiography shows
• • Aneurysm, stenosis, occlusion of vessels.

POLYARTERITIS Treatment

• Glucocorticoids
• Cyclophosphamide Plasma exchange.
• Prognosis is poor.

GIANT CELL ARTERITIS

• Also called temporai arteritis or cranial arteritis.
• Disease of, medium and large-sized arteries.
• Occurs at age more than 50 years, more common in females.

Clinical manifestations

• · Fever
• · Anaemia
• · Increased ESR
• · Headaches
• · Malaise
• · Weight loss
• · Sweating
• · Arthralgias
• · There is polymyalgia rheumatica-stiffness and
• pain of shoulders, back and hips
• · There is claudication
• · Strokes and MI can occur
• · Loss of vision or sudden blindness due to is­chemic optic neuropathy.
• · Death can occur due to cerebrovascular acci­dents, MI, aortic aneurysm.

GIANT CELL ARTERITIS Diagnosis

• · Increased ESR
• · Biopsy of temporal artery shows inflammation
• · Ultrasonography of temporal artery.

GIANT CELL ARTERITIS Treatment

• Prednisolone 40 – 60 mgjday.

TAKAYASU ARTERITIS

• Affects medium and large–sized arteries especially aortic arch and its branches.
• Also called aortic arch syndrome. Affects young girls and women.
• Aorta and pulmonary artery involved.
• Major branches of aorta at the origin are involved.
• There is panarteritis and infiltration with giant cells.

Clinical and Lab findings

• · There is fever, night sweats, arthralgias
• · There is decrease or absence of peripheral pulses
• · There are differences in blood pressure in different limbs
• · Arterial bruits
• · Subclavian artery is involved most commonly
• · There is hypertension
• · There are renal, cardiac, cerebral symptoms due to stenosis of arteries supplying them.

Arteriography

• · Irregular vessel walls
• · Stenosis with post stenotic dilatations
• · Aneurysm formation
• · Occlusion of arteries
• · Increased collatera I circu lation.
• Histopathology
• • Inflammation of vessels.

TAKAYASU ARTERITIS Treatment

• Death occurs due to congestive heart failure, cerebrovascular accidents, MI, rupture of aneu­rysm, renal failure
• Treatment includes glucocorticoid therapy 40-60 mg Prednisone per day
• Angioplasty of vessels involved
• Surgical correction
• Prosthetic grafts
• Methotrexate 25 mgjweek.

HENOCH-SCHONLEIN PURPURA

• It is a small vessel vasculitis, also called anaphy­lactoid purpura.
• Usually seen in children.
• There is palpable purpura on buttocks and extremi­ties.
• Arthralgias and Gastrointestinal symptoms.
• Glomerulonephritis with proteinuria, microscopic he­maturia, RBC casts in upto 50% of patients, resolves spontaneously.

Pathology

• · Antigens like drugs, upper respiratory tract in­fections, foods, insect bites, immunizations may cause the disease.
• · IgA antibody seen.

HENOCH-SCHONLEIN PURPURA Diagnosis

• · Skin biopsy showing vasculitis with IgA and C3 deposition
• · Renal biopsy rarely needed.

HENOCH-SCHONLEIN PURPURA Treatment

• Death is rare
• Glucocorticoids 1 mgjkgjday may be given.

IDIOPATHIC CUTANEOUS VASCULITIS

• It is a hypersensitivity vasculitis and cutaneous an­giitis.

IDIOPATHIC CUTANEOUS VASCULITIS Treatment

• Glucocorticoids, Methotrexate, Azathioprine.

ESSENTIAL MIXED CRYOGLOBULINEMIA

• Cryoglobulins are cold precipitable immunoglobulins. There is palpable purpura.
• Arthralgias, Neuropathy and Glomerulonephritis.

Treatment

• Glucocorticoids
• Plasmapheresis Cytotoxic agents.

COGAN’S SYNDROME

• There is interstitial keratitis with vestibuloauditory symptoms with aortitis and aortic valve disease.

COGAN’S SYNDROME Treatment

• Glucocorticoids.

KAWASAKI DISEASE

• Also called mucocutaneous lymph node syndrome.
• Acute febrile multisystem disease of children.
• Vasculitis of coronary arteries occurs.
• Aneurysm and thrombosis may occur.
• Pericarditis, Myocarditis, MI, Cardiomegaly. Prognosis is good.

KAWASAKI DISEASE Treatment

• Gamma globulins IV 2 gjkg single infusion in 10 hrs.
• Aspirin 100 mgjkgjday for 2 wks. followed by 5 mg/kg/day.

DRUG-INDUCED VASCULITIS

• Can occur with several drugs-Allopurinol, Sulphonamide, Penicillin, Thiazides etc.
• Fever, Malaise, Arthralgias and Skin involvement.

Treatment

• Stop the offending drug Glucocorticoids Cyclophosphamide.