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Rovner

VASCULITIS

Definition: Inflammation with necrosis of blood vessels resulting in variable clinical presentations depending upon:

      1. size of vessel
      2. type of vessel
      3. histological infiltrate

Pathophysiology:

Antigen-Antibody Complex Formation

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Immune Complex Deposition

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Complement Derived Chemotaxis

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PMN Lysosomal Enzyme Release

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Vessel Wall Injury, Necrosis

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VASCULITIS

  1. Vasculitis - Types

    1. Hypersensitivity Group (Leukocytoclastic)

      1. Vessel type: small, usually post capillary venules

      2. Symptoms: Skin-Palpable Purpura

        1. vesicles, bullae, ulceration

        2. urticaria (collagen vascular disease)

      3. Systemic:

        1. Joints--arthritis (50%)

        2. Renal--hematuria

        3. Other--GI tract

      4. Etiology:

        1. Infectious (Strep, viral, Rickettsial)

        2. Drug Reaction

        3. Collagen vascular disease

        4. Cryoglobulinemia

        5. Internal Malignancy

        6. Henoch-Schonlein Purpura

          1. Palpable purpura (dependent areas-legs and buttocks)

          2. Abdominal pain

          3. Arthritis

          4. Hematuria

          5. Increased IgA

        7. Idiopathic

      5. Laboratory work-up

        1. CBC, Platelets

        2. Urinalysis, BUN, Creatinine

        3. Chest x-ray

        4. ANA

        5. Cryoglobulins

        6. Serum Protein Electropheresis

        7. ESR (nonspecific)

      6. Differential Diagnosis:

        1. Thrombocytopenia

        2. Sepsis

      7. Treatment

        1. removal of offending agent

        2. steroids

        3. dapsone

    2. Polyarteritis Group (Periarteritis)

      1. Polyarteritis Nodosa

        1. Vasculitis of small-medium sized arteries with segmental (skip) involvement.

        2. Symptoms:

          1. Constitutional

          2. Renal--hypertension, glomerulonephritis

          3. Neuromuscular--arthritis, myalgia, sensorimotor neuropathy (mononeuritis multiplex)

          4. Gastrointestinal--functional

          5. Cutaneous--Livedo reticularis, Nodules (painful shiny red nodules in crops usually on lower extremities), Ulcerations

          6. Other--cardiac, CNS

        3. Laboratory Work-Up: Nonspecific

        4. Treatment:

          1. Steroids (50% 5 yr. survival)

          2. Cyclophosphamide (80% 5 yr. survival)

          3. None (10% 50 year survival)

      2. Churg-Strauss

        1. Allergic granulomatous angiitis

        2. Asthma, Eosinophilia

        3. Pulmonary vessel involvement--100%

        4. Splenic involvement--70%

    3. Granulomatous

      1. Wegener's Granulomatosis

        1. Granulomatous vasculitis of upper and lower respiratory tract, glomerulonephritis, and other small arteries

        2. Symptoms:

          1. Respiratory Tract--100%

          2. sinusitis, rhinitis, septal destruction, cough, dyspnea

          3. Renal--80% glomerulonephritis

          4. Cutaneous--40-50% nodules, ulcers

          5. Other--joints, CNS, cardiac

        3. Laboratory work-up

          1. ESR markedly elevated

          2. Chest x-ray (cavitary lesions, nodules)

        4. Treatment:

          1. 90% 2 yr. mortality without treatment

          2. cyclophosphamide and steroids

      2. Giant Cell Arteritis

        1. Temporal Arteritis (Polymyalgia Rheumatica)

        2. Giant cell infiltrate of large arteries (temporal, carotid)

        3. Severe, disabling musculoskeletal pain,

        4. Blindness without immediate steroids in high doses

        5. ESR extremely elevated (over 100)

  2. SYSTEMIC DISEASE

    1. Pruritus

      1. Thyroid (hyper- or hypo) TSH, T4

      2. Renal disease BUN, Creatinine

      3. Hepatic disease LFT's

      4. Malignancy (Hodgkins Disease) Chest x-ray

      5. Polycythemia vera CBC, platelets

      6. Diabetes glucose

    2. Endocrine

      1. Adrenal

        1. Addison's Disease

          1. Hypoadrenalism

          2. generalized bronzing

          3. dark pigmentation on gums

        2. Cushing's

          1. Hyperadrenalism

          2. moon facies

          3. buffalo hump

          4. hirsutism

          5. acanthosis nigracans

      2. Thyroid

        1. hyperthyroidism--pretibial myxedema, thyroid acropachy

        2. hypothyroidism--myxedema, alopecia, xerosis, macroglossia

      3. Diabetes Mellitis

        1. Necrobiosis Lipoidica Diabeticorum

        2. Diabetic Dermopathy

    3. Malignancy

      1. Pruritus

      2. Dermatomyositis

      3. Acanthosis Nigracans

      4. Acquired Ichthyosis

      5. malignant down ("lanugo hairs")

    4. AIDS

      1. Oral hairy leukoplakia--EB virus

      2. Kaposi's Sarcoma

      3. Sudden, severe Seborrheic Dermatitis

      4. Molluscum contagiosum-- refractory

      5. Unusual or opportunistic infections

      6. Bacillary angiomatosis

      7. Chronic cutaneous herpes virus infections
      8. Lipodystrophy