Vasculitis Syndrome Causes Differential Diagnosis Symptoms and Treatment

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 Syndrome 2

Vasculitis Syndrome Causes Differential Diagnosis Symptoms and Treatment


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 largesized 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.