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BY : 

BY SHASHIDHAR PATIL PG in Gen Medicine Basappa Memorial Hospital Mysore VASCULAR PURPURA

Purpura : 

Purpura Purpura and bruising, the main terms used to describe bleeding into the skin, may occur as isolated phenomena or as part of a systemic disorder. Purpura is the hallmark of vasculitis affecting the (usually smaller vessels of the) skin, and may be the dominant feature or a minor part of a systemic vasculitis

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Vasculitis typically causes other cutaneous lesions, including urticarial lesions, nodules, ulcers, livedo and frank necrosis, which have a purpuric component due to vessel wall damage. The same clinical pattern may arise from many different causes, both vasculitic and non-vasculitic, and the aetiology of either purpura or vasculitis may be impossible to determine.

Definition of purpura : 

Definition of purpura Purpura is discoloration of the skin or mucous membranes due to extravasation of red blood cells. Petechiae are small purpuric lesions usually 1–2 mm (occasionally up to 4 mm) across, often occurring in crops. Ecchymoses or bruises are larger extravasations of blood.

Clinical patterns of purpura : 

Clinical patterns of purpura Features that suggest a need for further investigation include: Larger or variably sized lesions, particularly when not in sun damaged skin Numerous or widespread lesions Lesions occurring in crops Palpable lesions Lesions forming reticulate patterns (livedo, retiform pattern) Associated features such as pustules, necrosis, nodules, splinter haemorrhages, etc. Evidence of bleeding from other sites, e.g. haematuria Associated general symptoms, e.g. fever, malaise, arthralgia.

Vasculitis: Basic Facts : 

Vasculitis: Basic Facts Vasculitis affects all ages, although some types are restricted to certain age groups Vasculitis tends to affect Caucasians, although many African-Americans are affected Vasculitis has a genetic component, but is not heritable Vasculitis is a chronic relapsing disease, although some patients experience prolonged remission

Vasculitis: Definition : 

Vasculitis: Definition Pathologist Inflammatory destruction of blood vessels Infiltration of vessel wall with inflammatory cells Leukocytoclasis Elastic membrane disruption Fibrinoid necrosis of the vessel wall Ischemia, occlusion, thrombosis Aneurysm formation Rupture, hemorrhage Rheumatologist A clinicopathologic process characterized by inflammatory destruction of blood vessels that results in occlusion or destruction of the vessel and ischemia of the tissues supplied by that vessel. “Systemic vasculitides”


VASCULITIS CAUSES ENVIRONMENTAL EXPOSURES Silica, Organic compounds, ? GENETIC PREDISPOSITION Family members with autoimmune diseases INFECTION Bacterial, Viral, Parasitical ? No clear proof yet although in some cases hepatitis B and C are associated with vasculitis

Vasculitis: Classification : 

Vasculitis: Classification Large-vessel vasculitis Giant cell arteritis, Takayasu’s arteritis Behcet’s disease, Cogan’s syndrome Medium-vessel vasculitis Polyarteritis nodosa Buerger’s disease, Central nervous system vasculitis, Kawasaki’s disease, Rheumatoid vasculitis Small-vessel vasculitis Wegener’s, microscopic polyangiitis, Churg-Strauss Cryoglobulinemic vasculitis, Henoch-Schönlein purpura,

Vasculitis: Classification : 

Vasculitis: Classification Large-vessel vasculitis Aorta and the great vessels (subclavian, carotid) Claudication, blindness, stroke Medium-vessel vasculitis Arteries with muscular wall Mononeuritis multiplex (wrist/foot drop), mesenteric ischemia, cutaneous ulcers Small-vessel vasculitis Capillaries, arterioles, venules Palpable purpura, glomerulonephritis, pulmonary hemorrhage

Summary of Vasculitides : 

Summary of Vasculitides

ANCA-associated vasculitides : 

ANCA-associated vasculitides Wegener’s granulomatosis: granulomatous inflammation involving the respiratory tract and necrotizing vasculitis affecting small to medium-sized vessels Microscopic polyangiitis: Necrotizing vasculitis affecting the small vessels. Churg-Strauss Syndrome: Eosinophil-rich and granulomatous inflammation involving the medium-sized vessels, and associated with asthma and eosinophilia

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Necrotizing Granuloma Hypereosinophilia Sinusitis Subglottic stenosis Pulmonary nodules Orbital pseudotumor Asthma Pulmonary infiltrates Myocarditis Pulmonary capillaritis Glomerulonephritis Sensory neuropathy Mononeuritis multiplex

Etiology : 

Etiology Ag-Ab complexes deposit in blood vessel walls whose integrity have already been compromised by vasogenic amines (histamine, leukotrienes, bradykinin) released from activated platelets and mast cells Complement components (primarily the classical pathway) are activated and recruit PMN’s

Etiology : 

Etiology Exactly why only certain immune complexes cause vasculitis and why only certain blood vessels are affected remains in large part a mystery

Hypersensitivity Reactions : 

Hypersensitivity Reactions Type I – immediate HS “classic” allergy Prior sensitization IgE mediated Massive degranulation Early and late responses Type 2 – cytotoxic HS IgG binds to target tissue C’ activation Direct cytotoxic action AIHA ITP Goodpasture’s Myasthenia Gravis

Hypersensitivity Reactions : 

Hypersensitivity Reactions Type 3 – IC HS “serum sickness” Ab form complex with circulating Ag Deposition results in C’ activation Leukocytoclastic vasculitis is hallmark manifestation Arthus reaction Type 4 – cell-mediated HS Delayed-type HS Previously sensitized T-cells are required Mantoux test Contact dermatitis Allograft rejection


ANCA Anti-Neutrophil Cytoplasmic Antibodies Ab directed against proteins in the cytoplasmic granules of PMN’s and monocytes Wegener’s Granulomatosis Microscopic Polyangiitis Churg-Strauss Crescentic/necrotizing GN

c-ANCA : 

c-ANCA Serum from patients bind to cytoplasmic granules and show a granular appearance on immunofluorescence Proteinase-3 (PR-3) is the major antigen Serine protease Present in azurophilic granules

C-ANCA staining pattern of ethanol-fixed normal human neutrophil : 

C-ANCA staining pattern of ethanol-fixed normal human neutrophil

p-ANCA : 

p-ANCA Localized, peri-nuclear staining pattern on PMN’s Myeloperoxidase (MPO) is the major target Ag Elastase Cathepsin G Lactoferrin Lysozyme Permeability-increasing protein Only MPO has been convincingly associated with vasculitis, the others may be seen in other ANCA+ diseases (IBD, drugs, endocarditis)

Proposed Mechanism of Disease : 

Proposed Mechanism of Disease PR-3 and MPO are mobilized to surface of PMN’s and monocytes when activated by TNF-α or IL-1 Now can react with circulating ANCA PMN’s degranulate and induce inflammation locally How the ANCA are generated in the first place is less clear





Vasculitis: Diagnosis : 

Diagnosis of a systemic vasculitis is often a diagnosis of exclusion, based on recognition of the clinical syndrome e.g. Churg-Strauss: adult onset asthma x 2 years, followed by atypical pneumonias, followed by peripheral nerve involvement Biopsy of involved organ is the most straightforward method of establishing a diagnosis Biopsy may be helpful to exclude infection/malignancy Other tests may be suggestive, but not diagnostic ESR, CRP CT: pulmonary hemorrhage, cavitary lesions Bronchoscopy: pulmonary hemorrhage (hemosiderosis) Urinalysis: for patients with kidney vasculitis ANCA (antineutrophil cytoplasmic antibodies) Angiogram (including MRA, CT-angiogram) Vasculitis: Diagnosis

Approach to patient with vasculitis : 

Approach to patient with vasculitis Characteristic clinical patterns Tissue biopsy Establish diagnosis Patient with a multi system disorders (suspected vasculitis) Angiography Supporting laboratory testing Look for underlying dis. Look for offending antigen Specific vasculitis syndrome No No Yes Yes Treat vasculitis Remove antigen TTT disease Follow up No further action Syndrome resolved Syndrome resolved No No

Vasculitis: Treatment : 

Remission induction: Cyclophosphamide 2mg/kg po qd x 3-6 months [or 15 mg/kg IV q 2 wk x3 then q 3 weeks x 6-12 months] Prednisone 1mg/kg po qd x 1 month, then taper [Bactrim, Calcium, Vitamin D] Remission maintenance (minimum 2 years) Methotrexate 20-25 mg po q week + folate Azathioprine 2mg/kg po qd Mycophenolate mofetil 1.5 g po BID Leflunomide 20-30 mg po BID Vasculitis: Treatment

Vasculitis: Monitoring : 

Vasculitis: Monitoring Large-vessel vasculitis MRI/MRA chest/abdomen/pelvis every 6-12 months Medium-vessel vasculitis Mesenteric angiogram to assess disease activity EMG/NCV to monitor nerve damage Wound care for cutaneous ulcers Small-vessel vasculitis Chest CT every 6-12 months Blood and urine tests every 1-4 weeks

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European Vascualitis Study Group(EUVAS)

Vasculitis: Burden of Disease : 

Patient with Wegener’s granulomatosis Drugs: cyclophosphamide, prednisone, TMP/SMX, Calcium+vitamin D Monitoring: Lab tests every 1-4 weeks CT scans every 6-12 months PFTs with flow-volume loops, EMG/NCV Consultants: Otolaryngology: Sinusitis, subglottic stenosis Nephrology: Chronic renal insufficiency Gynecology: Fertility counseling Neurology: Management of peripheral neuropathy Rehabilitation, pain management Vasculitis: Burden of Disease

Small- Vessel Vasculitides : 

Small- Vessel Vasculitides

Wegener’s : 

Wegener’s Systemic disease Granulomatous vasculitis of upper and lower respiratory tract with associated GN, variable degrees of disseminated vasculitis Involves small arteries and veins Prevalence: 3 per 100,000 Extremely rare in blacks Equal M:F ratio Mean age of onset is 40

Wegener’s : 

Wegener’s Multiple b/l nodular cavitary infiltrates Lung biopsy: classic granulomatous necrotizing vasculitis Nasal ulcers, sinus disease, septal perforation, saddle-bridge deformity, tracheal stenosis Nasal biopsy: usually reveals the same, not as sensitive FSGS  RPGN Kidney biopsy: rarely shows granulomas or immune complex deposition (focal, segmental, necrotizing pauci-immune GN)

Wegener’s : 

Wegener’s Peripheral WBC tests indicate an unbalanced TH1 cytokine pattern 90% are PR-3 positive (c-ANCA) during active disease A few pt’s will be MPO positive rather than PR-3 BUT… one must establish tissue diagnosis!! Aim for lungs. Must differentiate from other rare diseases such as angiocentric immunoproliferative diseases and lymphomatoid granulomatosis

Focal or diffuse necrotizing extracapillary glomerulonephritis is the histological hallmark of ANCA-associated Vasculitis : 

Focal or diffuse necrotizing extracapillary glomerulonephritis is the histological hallmark of ANCA-associated Vasculitis

Massive necrosis is usually associated to diffuse circumferential extracapillary proliferation. From a clinical point of view, the patient is affected by rapidly progressive renal failure. : 

Massive necrosis is usually associated to diffuse circumferential extracapillary proliferation. From a clinical point of view, the patient is affected by rapidly progressive renal failure.

Focal glomerulonephritis with crescent formation on renal biopsy specimen, characteristic of Wegener granulomatosis : 

Focal glomerulonephritis with crescent formation on renal biopsy specimen, characteristic of Wegener granulomatosis Crescent formation

The biopsy specimen of a lung from a patient with Wegener granulomatosis showing evidence of vasculitis and inflammation : 

The biopsy specimen of a lung from a patient with Wegener granulomatosis showing evidence of vasculitis and inflammation

Wegener’s Rx : 

Wegener’s Rx Previously, uniformly fatal in matter of weeks to months Cyclophosphamide (2mg/kg/day) Maintain WBC >3000 & PMN >1500 6-12 months!! Glucocorticoids 1mg/kg/day, taper at 1 month and off at 6 months Remission rate: 75% 90% have marked improvement Plasmapheresis in refractory/progressive cases

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The most common hypersensitivity vasculitis of childhood & young adults. Preceded by an upper respiratory tract infection, but the etiology ? unknown. Boys and girls are affected equally. The median age of onset is 4 years. It follows a self-limiting course in most patients. Henoch-Schonlein purpura

Clinical presentation : 

Clinical presentation The classic triad is palpable purpura with a normal platelet count, colicky abdominal pain, and arthritis + fever. Purpura appears first on lower extremities & dependent areas and buttocks. Arthritis is transient and usually involves the knees and ankles. Hemoptysis in up to 1/3 of patients . Half have occult gastrointestinal bleeding. 10-50% has renal involvement, from transient microscopic hematuria to RPGN.

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Made on clinical grounds + skin biopsy. Laboratory results are variable (ESR, complement, immune complexes. & IgA). Diagnosis Largely supportive = hydration & monitoring. NSAIDs for joint pain. Corticosteroids 10-30 mg/d for abdominal pain, edema, and nephritis. Treatment

Microscopic Polyangiitis : 

Microscopic Polyangiitis Disease entity characterized by a necrotizing vasculitis with few or no immune complexes, affecting small vessels (arterioles, capillaries, venules)

Microscopic Polyangiitis : 

Microscopic Polyangiitis Disease entity characterized by a necrotizing vasculitis with few or no immune complexes, affecting small vessels (arterioles, capillaries, venules) First coined in 1992, by the Chapel Hill Consensus Conference on the Nomenclature of Systemic Vasculitides Incidence is uncertain due to previous lumping together with PAN. Etiology unknown.

Microscopic Polyangiitis : 

Microscopic Polyangiitis Pauci-immune GN is very common (79%) renal biopsy is identical to that in Wegener’s Pulmonary infiltrates and hemorrhage may occur as a result of capillaritis, but biopsies lack granulomas Upper airway disease and pulm nodules/cavities absent Immunohistochemical staining lacks IC deposition, suggesting that IC complex formation is not part of the pathogenesis

Microscopic Polyangiitis : 

Microscopic Polyangiitis 75% of patients are c-ANCA+ Treatment is identical to Wegener’s, and distinguishing between the two is some what academic purpose 5-yr survival is 74%

Churg-Strauss Syndrome : 

Churg-Strauss Syndrome Incidence: ~1 per 1,000,000 Occurs at any age, but mean is 48yrs Clinical Tetrad Asthma Eosinophilia (blood and peripheral) Extravascular granuloma Vasculitis affecting mutiple organs Involves small and medium-sized arteries, capillaries, and veins

Churg-Strauss Syndrome : 

Churg-Strauss Syndrome Granulomatous inflammation with eosinophilic infiltration of involved organs Lung Kidney Skin Heart Presents as systemic syndrome (fever, malaise, anorexia, weight loss) with severe asthma attacks (may be precipitated by initiation of leukotriene inhibitors)

Churg-Strauss Syndrome : 

Churg-Strauss Syndrome p-ANCA sensitivity: 48% Treatment Glucocorticoids titrated to control asthma Add cyclophosphamide as second line as in Wegener’s

Goodpasture’s Syndrome : 

Goodpasture’s Syndrome A specific subset of anti-GBM disease in which pulmonary hemorrhage occurs Target: noncollagenous domain of α3 chain of Type IV collagen Typically seen in young males (5-40y/o) Type II HS reaction - inflammation and tissue destruction mediated by direct Ab binding and secondary activation of classical C’ pathway or direct activation of cytotoxic T-cells or phagocytes

Goodpasture’s Syndrome : 

Goodpasture’s Syndrome Kidney RPGN and crescentic GN are most common Pulmonary hemorrhage (50-60% of all pt’s), quite rare in patients >50y/o NOT associated with ANCA positivity

Goodpasture’s Syndrome : 

Goodpasture’s Syndrome Gold standard diagnostic test? Renal biopsy with immunohistochemical staining Circulating anti-GBM antibodies (IgG) is 90-95% sensitive Levels directly correlate with severity, organ survival, and relapse Treatment Plasmapheresis daily until titers undetectable (1-2 weeks!) Prednisone 1mg/kg/day Cyclophosphamide or azathioprine

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Medium-sized vessel disease

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Etiology: Unknown; some cases have HBV infection. PAN is twice commoner in males, around 40s. Constitutional symptoms: malaise, fever, weight loss & musculo-sckeletal pain. Polyarteritis nodosa (PAN)

Clinical presentation : 

Clinical presentation Kidneys are the most commonly involved organs, GN & renal failure. Coronary arteritis with angina or MI. Pericarditis is common. GIT - abdominal pain, bleeding, and bowel obstruction or perforation. Rupture of mesenteric aneurysm intraperitoneal hemorrhage. Peripheral neuropathy: arteritis of vasa nervosa- painful mononeuritis multiplex. Vasculitis of CNS - encephalopathy, convulsions, +/- CVS.

Clinical presentation cont. : 

Clinical presentation cont. Cutaneous lesions: vascular purpura, livedo reticularis, peripheral gangrene & skin nodules. Myalgias & arthralgias. Orchitis & epididymitis Gangrene Livedo reticularis

Laboratory studies. : 

Laboratory studies. ESR, CRP, WBCs, Platelets are usually high. Anemia, hematuria & proteinuria. Hypocomplementemia. Positivity for ANCA is rare in PAN. Hepatitis B surface antigen (HBsAg) (>30%). Angiography often shows microaneurysms and stenoses in medium-sized vessels. Biopsy is useful to confirm diagnosis.

Treatment : 

Treatment PAN without hepatitis B: High-dose corticosteroids (40 to 60 mg/d). 1-2mg/kg Cyclophosphamide is added for severe cases = 2mg/kg/d, then tapered. In hepatitis B-related PAN = antiviral (Lamivudine (zevix) or Interferon), , plasma exchange + corticosteroids Relapses are rare.

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Mucocutaneous LN syndrome Etiology: unknown. Acute febrile disease Affecting infants & children < 5 Yrs. Kawasaki disease

Clinical presentation : 

Clinical presentation The onset is abrupt, with high fever1-2 W. +Painful cervical lymphadenopathy. Bilateral conjunctival congestion. Dryness, redness, and fissuring of the lips "strawberry" tongue. Exanthema of the trunk Redness of the palms and soles /desquamation. Carditis + heart murmurs + ECG changes. CAD + dilatation or aneurysms. Abdominal pain, vomiting, diarrhea. Arthritis

Treatment. : 

Treatment. Supportive in uncomplicated cases. Echocardiography to detect CAD. +: high-dose IV gamma globulin: IVI 2 g/kg. Low-dose aspirin (3 to 5 mg/kg daily). Follow-up coronary angiography.

Mechanisms of action of IV gamma globulin : 

Mechanisms of action of IV gamma globulin Decreases expression of adhesion molecules on endothelial cells Binds to inflammatory cytokines Decrease number of activated T lymphocytes (-CMI). Blocking antibody binding sites.

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Large vessel disease

Giant-Cell Arteritis &Polymyalgia Rheumatica : 

Giant-Cell Arteritis &Polymyalgia Rheumatica

GCA : 

GCA Chronic vasculitis of medium and large vessels More common at age>50, incidence peaks at 70-80 Affects women more often than men, 2:1

Epidemiology : 

Epidemiology Polymyalgia Rheumatica & Giant-cell Arteritis Generally affect people over 50 One patient may have both 50% with GCA have PMR 10% with PMR have GCA Incidence GCA: 18 cases per 100,000 over 50 Men: 8/100,000 Women: 24/100,000 PMR: 500 cases per 100,000 over 50

Pathophysiology : 

Pathophysiology Vasculitis of extracranial branches of aorta, spares intracranial branches Transmural inflammation-> intimal hyperplasia-> luminal occlusion Symptoms are due to end-organ ischemia

Histology : 

Histology Granulomatous inflammation with giant cells in junction between intima and media. (50%) Inflammatory infiltrate usually focal and segmental

Etiology : 

Etiology Likely influenced by multiple genetic and environmental factors No evidence of autoantibodies Cellular immune response involving T-cells, APCs, macrophages

Presentation : 

Presentation Onset usually gradual, but may be abrupt Most frequent symptom: headache (2/3) Usually accompanied by syndrome of systemic inflammation: fever, malaise, weight loss, anorexia (40%) Frequently associated with polymyalgia rheumatica (39%)

Symptoms : 

Symptoms Headache 68% Jaw claudication 45% Transient visual symptoms 16% Fixed visual symptoms 14% CNS Abnormalities 15% Swallowing claudication/ dysphagia 8% Tongue claudication 6% Limb claudication 4%

Clinical Signs : 

Clinical Signs Weight loss or anorexia 50% Decreased temporal artery pulsations 46% Fever 42% Artery tenderness 27% Erythematous or swollen scalp arteries 23% Large artery bruits 21 % Fundoscopic abnormalities 18%

Clinical Features of PMR : 

Clinical Features of PMR History Consitutional symptoms: Fever, night sweats, depression, malaise Pain and stiffness in shoulder and/or pelvic girdle Morning stiffness of 45 minutes or more Difficulty getting up, doing ADLs

Clinical Features of PMR : 

Clinical Features of PMR Exam Affected muscle groups TENDER but not WEAK Laboratory investigations ESR > 40 mm/hour

Laboratory Findings : 

Laboratory Findings Erythrocyte sedimentation rate > 50 22% of patients with biopsy-proven GCA have normal ESR Therefore, normal ESR does NOT rule out GCA Mild-moderate anemia of chronic disease Elevated LFTS (1/3)

Diagnosis : 

Diagnosis Temporal artery biopsy should be performed in all patients with suspected GCA Sensitivity of unilateral biopsy: 88% Negative Predictive Value: 91% Bilateral biopsies increase sensitivity, may be prudent

Diagnosis: Imaging : 

Diagnosis: Imaging MRI/MRA may be used to diagnose large-vessel GCA, especially with no cranial arteritis. Also may held guide biopsy by localizing inflammation Other possible imaging modalities include arteriography, ultrasound, and PET.

Treatment : 

Treatment Glucocorticoids are the established treatment Initial dose: 40-60 mg prednisone daily Slow taper after 2-4 weeks if symptoms controlled Prolonged treatment often required to avoid relapse IV pulse methylprednisolone (1000 mg IV daily x 3 days) if vision loss present

Treatment : 

Treatment Data on methotrexate is mixed; may moderately reduce steroid dependence Infliximab does not reduce incidence of relapse Anti-platelet agents reduce ischemic events-> low dose aspirin is recommended

Treatment : 

Treatment If clinical suspicion is high, treatment should NOT be delayed for biopsy Temporal artery biopsies should be performed as soon as possible after treatment is initiated, but biopsy results should not be affected for several weeks

Takayasu’s Arteritis(pulseless disease) : 

Takayasu’s Arteritis(pulseless disease) Vasculitis of large arteries ,mainly arch of aorta, characterized by thickening and obliteration of the mouths of great vessels. Symptoms are secondary to luminal narrowing More common in females younger than 40 years old Course of disease is variable ,may enter in quiescent stage.

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Takayasu’s Arteritis Chronic inflammatory disorder, affecting the aorta & its major branches. Affects women mainly (f:m = 9:1), Ages 15 - 25.

It has two phases:- : 

It has two phases:- * Early systemic phase: Malaise, fever, night sweats, weight loss, myalgias, and arthralgias. * Later occlusive phase: Claudicating pain (arms & neck), headaches, syncope, and visual disturbances, + aortic regurgitation

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Clinical presentation Arterial bruits & absence of pulses. Hypertension is common but is often lower in the arms. Symptoms of vascular insufficiency (claudication, TIA, and syncope) Bruits, weak pulses, and discrepancies of limb blood pressure in young women

Laboratory studies : 

Laboratory studies High ESR in the early phase. Chest X-ray: Widened aortic shadow, Irregularity of the descending aorta, Cardiac enlargement, Hilar fullness. Arteriography: is most helpful. MRI: thickened and altered aorta

Treatment : 

Treatment Oral corticosteroids.. 1 mg/kg daily, with a maintenance 5 to 10 mg/d. Cytotoxic drugs in patients failing steroid treatment. Surgery and angioplasty: in advanced disease.

Thromboangiitis obliterans(Buerger’s Disease) : 

Thromboangiitis obliterans(Buerger’s Disease) This condition is marked by segmental,thrombosing,acute and chronic inflammation of intermediate and small arteries & veins in the limbs with extension to accompanying nerves. Typically seen in heavy smokers males before the age of 35. Intermittent claudication followed by pain at rest,might end in gangrene. Etiology ? Endothelial injury by toxins in tobacco.

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A child complaining of : 

A child complaining of Arthritis Palpable purpuric eruptions in LL Abdominal pain & GI bleeding + Hemoptysis After upper RTI = ??? 1

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A history of asthma, allergic rhynitis, atopy, peripheral neuropathy, cutaneous eruptions, pericarditis, cardiomyopathy, myocardial infarction hypereosinophilia may suggest ??? 2

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CSS Churg-Strauss syndrome

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patient over the age of 50 recent onset of headache, Jaw claudication scalp tenderness, loss of vision, myalgias, fever (FUO), a high ESR, anemia. ???? 3

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Recurrent oral ulcers, > 3 times in 1yr. Genital ulcer or scare, Uveitis, cells in vitreous, retinal vasculitis, Superficial thrombophlebitis, Erythema nodosum, Papulopustules Pathergy (2mm eryth- 1-2days-25g-5mm depth).????????? 4

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Purulent or bloody nasal drainage, Nasal mucosal ulceration, Saddle nose deformity Otitis media . Cough, dyspnea, hemoptysis Massive pulmonary hemorrhage Hematuria, Proteinuria, CRF Fever, weight loss, Cutaneous purpura, peripheral neuropathy, arthralgia/arthritis.??? 5

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A child <5yrs. complaining of: Arthritis, Acute onset of high fever, Bilateral conjunctival congestion, "strawberry" tongue. Painful cervical LN, Exanthema of the trunk, Carditis + heart murmurs & IHD Abdominal pain, vomiting & Diarrhea? 6

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A male, around 40s complaining of: Myalgias, arthralgias, fever, Sudden onset of sever HPT, LL swelling & renal impairment, Chest pain, dyspnea on exertion. Abdominal pain, bleeding, and bowel obstruction , abdominal collection. PN, painful mononeuritis multiplex, seizures, strocks. Palpable purpura, urticaria, livedo reticularis, peripheral gangrene and skin nodules. Orchitis and epididymitis. 7

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young women complaining of: Myalgias, arthralgias Claudication, Transient visual disturbances, Syncope Bruits, weak pulses, Discrepancies of limb blood pressure (LL>UL), 8

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