Vasculitides

Question 1

Clinical features of Polyarteritis nodosa

Answer 1

Sudden sharp pain in peripheral nerve distribution, later with weakness (foot drop). Cutaneous manifestations: palpable purpura, infarctive ulcers, Livedo reticularis, Digital tip infarctions, Livedo Reticularis, Mononeuritis Multiplex, Cutaneous Nodules

Question 2

Management of Polyarteritis nodosa

Answer 2

Treatment: High dose steroids (1 mg/kg/day), Immunosuppressives, such as cyclophosphamide or azathioprine, Plasma exchange; Treat viral hepatitis if present.

Question 3

Polyarteritis nodosa

Answer 3

Skin, peripheral nerves, joints, intestinal tract most commonly involved. Lungs spared. Uncommon- incidence 5 to 10 per million per year, Twice as common in men. ……. Assoc w/ Hep B (more commonly prior to vaccine)
Can affect myriad of organs (GU, abd, ocular), Musculoskeletal – arthralgias&raquo_space; synovitis, “Mononeuritis multiplex” >50%.

Question 4

Pathology of Hypersensitivity vasculitis (LCV)

Answer 4

Extravasated RBCs, fibrinoid necrosis

Question 5

Clinical features of Hypersensitivity vasculitis (LCV)

Answer 5

Pts with LCV complain of: itching, burning sensation
pain, asymptomatic lesions …………… Exam: Palpable purpura most common, Lesions usually round 1-3 mm diameter, May coalesce, plaque like, May ulcerate

Question 6

Management of Hypersensitivity vasculitis (LCV)

Answer 6

Treat the cause (if you can find it!); Generally results in rapid clearing in 2 wks. Treat chronic disease that primarily involves the skin with nontoxic modalities. Try to minimize systemic steroids, Avoid immunosuppressives. Colchicine or dapsone (inhib neutrophil fxn)

Question 7

Hypersensitivity vasculitis (LCV)

Answer 7

Leukocytoclastic vasculitis (LCV) - histopathologic term denoting small-vessel vasculitis.“Cutaneous vasculitis” “LCV” “Hypersensitivity vasculitis: all terms are used interchangeably. Many possible causes exist for this condition, but a cause is not found in as many as 50% of patients . The most commonly identified cause is a drug reaction ……. Various infections such as Hep C. Collagen Vascular Diseases (RA, Sjogrens, Lupus). Inflammatory Bowel Disease. Malignancy accounts for less than 1% of cases of cutaneous vasculitis.

Question 8

Cryoglobulinemia

Answer 8

Cryoglobulins – immunoglobulins & complement. Precipitation of blood proteins at temps <37C, Dissolve upon warming – tend to see peripheral involvement

Question 9

Etiology of Cryoglobulinemia

Answer 9

Chronic immune stimulation, defective clearance. Associated with hepatitis B & C

Question 10

Clinical features of Cryoglobulinemia

Answer 10

Prognonis is good, 70% at 10 yrs. Severe manifestations:

Glomerulonephritis , tends to be assoc Hep C, GI (abd pain, bleeding), Rapidly progressive neuropathy, CHF

Question 11

Management of Cryoglobulinemia

Answer 11

Labs: + cryoglobulins, Diminished complement, +RF, Hepatitis B & C, Renal failure, Lymphoproliferative d/o……….
Treatment: tx underlying hepatitis, malignancy, Prednisone (tapered rapidly), Rituxan if severe.

Question 12

Labs and Imaging of Wegner’s Granulomatosis

Answer 12

Renal: increased Scr, proteinuria, hematuria, red blood cell casts; + c-ANCA PR3 80-90%, most often with active disease; Anemia; Elevated inflammtory markers; Chest CT - nodules and infiltrations that often cavitate

Question 13

Diagnosis of Wegner’s Granulomatosis

Answer 13

Suspect with upper or lower respiratory tract involvement and nasal/oral ulcerations; PR3 positive c-ANCA; Biopsy of involved tissues - necrotizing granulomatous inflammatory process

Question 14

Clinical Manifestations of Wegner’s Granulomatosis

Answer 14

Nonspecific findings- fever, malaise, weight loss, arthralgias, myalgias, chronic rhinitis or worsening sinusitis. Pain over sinus areas. ………… Purulent or bloody nasal discharge. Nasal or mucosal ulcerations. Saddle-nose deformity …….. Pulmonary: chest pain, dyspnea, hemoptysis, pulmonary hemorrhage, Tracheal lesions and subglottic involvement -> stenosis. ………. Eye- uveitis, episcleritis, proptosis

Question 15

Management of Wegner’s Granulomatosis

Answer 15

High dose steroids (1mg/kg or IV 1g/d x3d). Immunosuppressives, such as Cyclophosphamide or Rituximab. Diffuse alveolar hemorrhage – plasmapharesis
Relapses common, maintenance therapy with less toxic drugs (methotrexate or azathioprin)

Question 16

Giant Cell Arteritis (temporal arteritits)

Answer 16

Granulomatous arteritis of the aorta and its major branches - extracranial branches of the carotid artery; patients > 50 years old, associated polymyalgia rheumatica

Question 17

Pathology of Giant Cell Arteritis (temporal arteritits)

Answer 17

Relatively common vasculitis: Onset > 50 yrs (avg age ~70 yrs), Male:female ratio: 1:4. Strong assoc PMR (prox pain & stiffness)

Question 18

Clinical Features of Giant Cell Arteritis (temporal arteritits)

Answer 18

Nonspecific symptoms: fatigue, low-grade fever, weight loss. Headache is the most common symptom (classically over temporal regions), Jaw claudication ……….. Scalp tenderness over arteries, esp Temporal. Impaired vision (most common neurologic sign (40% of cases)); ischemic optic neuritis can result in blindness pale, swollen disc on ophthalmologic exam. [diplopia]. New carotid artery bruits, or bruits over axillary/brachial arteries. Temple Artery Induration

Question 19

Management of Giant Cell Arteritis (temporal arteritits)

Answer 19

Treatment - high dose steroids (1 mg/kg). Given high stakes, Initiation of steroids should NOT be delayed until biopsy is done. Diagnostic utility of biopsy declines with each day on steroids

Question 20

Takayasu’s Arteritits

Answer 20

Granulomatous inflammation of the aorta & major branches, patients <40 years old

Question 21

Pathology of Takayasu’s Arteritits

Answer 21

Similar to GCA in presentation, histopathology, and lg vessel involvement but differs epidemiologically. Most frequent in young Asian women. Proximal aorta and branches most common.

Question 22

Clinical Manifestations of Takayasu’s Arteritits

Answer 22

Symptoms: dizziness, amaurosis, diplopia, angina (narrowing of coronary ostia), arm claudication, leg claudication, intra-abdominal ischemia (mesenteric arteries), cerebral ischemia (cervical arteries)……….. Physical: Asymmetric blood pressure >10 mm/Hg in nearly all pts. Hypertension (40%)- renal artery stenosis. Bruits- carotid, subclavian, etc. Aortic valve insufficiency (aortic root dilatation).

Question 23

Management of Takayasu’s Arteritits

Answer 23

Labs: Normochromic anemia, Elevated ESR; EKG- may have ischemia; CXR- may have widening of thoracic aorta; Imaging- Arteriography. ……….. Treatment: prednisone 1 mg/kg/day & immunosuppressive.

Question 24

Dx of LCV

Answer 24

Age>16 years at disease onset, Medication at disease onset, Palpable Purpura, Maculopapular rash, Biopsy -arterioles and venuoles with granulocytes in perivascular space with extravascular RBCs and fibrinoid necrosis

Question 25

GPA (Wegener’s Granulomatosis)

Answer 25

Triad- necrotizing granulomatous vasculitis of upper respiratory tract (sinuses), lower respiratory tract (lungs), and focal segmental glomerulonephritis (kidneys). Cutaneous, ocular, musculoskeletal, peripheral nervous systems commonly affected. Typically young or middle-aged adults. More common in men

Question 26

Dx of Polyarteritis Nodosa

Answer 26

Diagnosis: suspect with unexplained fever, weight loss, fatigue, and multisystem findings. Biopsy of clinically involved tissues: Transmural necrotizing inflammation. Imaging: MRA, angiograms. Multiple arterial aneurysms. Micro-aneurysms on angiography, infarctions on MRA
Mesenteric angiogram showing multiple areas of narrowing and aneuryms.

Question 27

Labs for Polyarteritis Nodosa

Answer 27

Labs: normochromic, normocytic anemia, elevated ESR
elevated LFTs, Hepatitis B surface antigen, Hepatitis C, A also found …….. Renal insufficiency but UA lacks RBC casts due to lack of glomerular inflammation.

Question 28

Livedo Reticularis in PAN

Answer 28

When due to vasculitis, does not blanch with pressure, and implies injury to vessel with extravasation of blood

Question 29

Mononeuritis Multiplex in PAN

Answer 29

Bilateral foot drop- common peroneal nerve injured as result of vasculitis within vasa nervorum

Question 30

Kawasaki’s Disease

Answer 30

Arteritis of large, medium, and small arteries but most notably coronaries, associated with the mucocutaneous signs & lymphadenopathy in children.

Question 31

Labs/Tests of Giant Cell Arteritis

Answer 31

Laboratory results: Elevated ESR ~100 mm/hr; Normocytic hypochromic or normocytic normochromic anemia. Other elevated acute phase reactants (crp, ferritin). Abnormal hepatic enzymes (20-30% pts). ……….. Biopsy of Temporal Artery: Positive 60-80% pts; Skip lesions, Biopsy contralateral side increases yield (Need 3-5 cm tissue), Granulomatous inflammation, disruption of internal elastic lamina & characteristic multinucleate giant cells

Question 32

Arteries commonly affected by Giant Cell Arteritis

Answer 32

Temporal, Occipital, Opthalmic, Facial and Lingual