Vasculitis Syndrome Causes Differential Diagnosis Symptoms and Treatment

Vasculitis Syndrome

Vasculitis is a diseasePneumothorax Symptoms Causes Diagnosis Treatment with emergency. Read more ... » 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 DiagnosisPneumothorax Symptoms Causes Diagnosis Treatment with emergency. Read more ... » 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 DrugPneumothorax Symptoms Causes Diagnosis Treatment with emergency. Read more ... »-induced vasculitis.

Mechanism of Vasculitis:

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

Vasculitis Syndrome PathophysiologyAcute Myocardial Infarction (AMI) Causes PATHOPHYSIOLOGY and Etiology. Read more ... » –

• 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.
• · RenalDiabetic Nephropathy and Renal complications of DM (Diabetes Mellitus). Read more ... » biopsy shows immune glomerulo-nephri­tis.
• · Positive anti proteinase 3 ANCA.

WEGENER’S GRANULOMATOSIS  Treatment

• GlucocorticoidsAdrenal Gland Steroids. Read more ... »
• 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, heartPRESENTATIONS OF ischemic heart disease(IHD). Read more ... », kidneyDiabetic Nephropathy and Renal complications of DM (Diabetes Mellitus). Read more ... », pe­ripheral nervous systemPneumothorax Symptoms Causes Diagnosis Treatment with emergency. Read more ... », gastrointestinal tract.

CHURG-STRAUSSSYNDROME Symptoms

• FeverThyrotoxic crisis or Thyroid storm. Read more ... », 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, headacheHeadache. Read more ... », abdomi­nal pain, myalgias
• · Renal involvement leading to hypertensionHyperthyroidism or THYROTOXICOSIS Causes, Symptoms and Diagnosis. Read more ... » 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 claudicationCYANOTIC CONGENITAL HEART DISEASE Coarctation of the Aorta with Clinical features. Read more ... »
• · Strokes and MI can occur
• · Loss of vision or sudden blindnessOphthalmologic Complications of DM (Diabetes Mellitus). Read more ... » due to is­chemic optic neuropathy.
• · Death can occur due to cerebrovascular acci­dents, MI, aortic aneurysmAORTIC ANEURYSM THORACIC AORTIC ANEURYSM and ABDOMINAL AORTIC ANEURYSM Etiology Symptoms with Treatment. Read more ... ».

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 pressureChronic Long-term Complications Of Diabetes Mellitus. Read more ... » in different limbs
• · Arterial bruits
• · Subclavian artery is involved most commonly
• · There is hypertension
• · There are renal, cardiacPRESENTATIONS OF ischemic heart disease(IHD). Read more ... », 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 failureHeart Failure Causes Symptoms NYHA classification with Heart Failure Treatment. Read more ... », 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.
• AcutePneumothorax Symptoms Causes Diagnosis Treatment with emergency. Read more ... » febrile multisystem disease of children.
• Vasculitis of coronary arteries occurs.
• Aneurysm and thrombosis may occur.
• PericarditisCOMPLICATIONS OF Acute Myocardial Infarction (AMI) AND TREATMENT -2. Read more ... », MyocarditisMYOCARDITIS Physical Examination Symptoms Clinical Manifestations Diagnosis. Read more ... », 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.