Dr. Müge Bıçakçıgil Kalaycı Vasculitis Dr. Müge Bıçakçıgil Kalaycı

Vasculitis A heterogenous group of clinical syndromes characterized by inflammation of blood vessels The clinical picture is essentially dependent on the size and extent of vessel involvement

Vasculitis Ambiguity of clinical presentations Limited diagnostic tests Difficulty in obtaining diagnostic tissue Therefore, difficult to diagnose AND classify

Incidence of Vasculitis Variable because of definitions Kawasaki seen almost exclusively in pediatric population Most other vasculitides in the fifth decade of life

All blood vessels can be affected from the largest (Aorta) to the smallest blood vessels in the skin (capillaries) Blood Vessel Injury Increased permeability Weakening (Aneurysm +/-Hemorrhage) Intimal proliferation and thrombosis, obstruction and local ischemia

What is Vasculitis?

Classification of Vasculitis Vasculitis may be classified by: The size and type of vessel involvement The histopathologic features (leukocytoclastic, granulomatous vasculitis, etc.) The pattern of clinical features

Classification of Vasculitis Important to classify the vasculitis since some types may be self-limited while others may be chronic However, initially it is important to determine the amount and extent of organ system involvement

Classification of Vasculitis Primary Vasculitis Syndromes Wegener's granulomatosis Churg-Strauss syndrome Polyarteritis nodosa Microscopic polyangiitis Giant cell arteritis Takayasu's arteritis Henoch-Schönlein purpura Idiopathic cutaneous vasculitis Essential mixed cryoglobulinemia Behçet's syndrome Isolated vasculitis of the central nervous system Cogan's syndrome Kawasaki disease Secondary Vasculitis Syndromes Drug-induced vasculitis Serum sickness Vasculitis associated with other primary diseases Infection Malignancy Rheumatic disease

Classification of Vasculitis Large Vessel Takayasu arteritis Giant Cell Arteritis Medium Vessel PAN Kawasaki’s Isolated CNS vasculitis Small Vessel Churg-Strauss Wegener’s Microscopic Polyangiitis HSP Essential Cryoglobulinemia Hypersensitivity vasculitis Vasculitis 2nd to CTD Vasculitis 2nd to viral infection

CLASSIFICATION TREE Vasculitis Large Blood Vessel Temporal Arteritis Takayasu Arteritis Small Blood Vessel Medium Blood Vessel Polyarteritis Nodosa Kawasaki’s Disease ANCA Associated Wegener’s Granulomatosis Churg-Strauss Vasculitis Microscopic Polyangiitis Drug Induced Non-ANCA Associated Immune Complex Hypersensitivity Vasculitis Cryoglobulinemic Vasculitis CTD related Vasculitis Henoch Schonlein Purpura Behcet’s Miscellaneous Paraneoplastic Vasculitis Inflammatory Bowel Disease

LARGE VESSEL VASCULITIS Giant Cell Arteritis (Temporal Arteritis) Takayasu’s Arteritis

Temporal (Giant-Cell) Arteritis Chronic granulomatous vasculitis affecting large arteries in older people Inflammation of the walls of large arteries Cranial arteritis (most common): Temporal, occipital, ophthalmic Subclavian, iliac/femoral Aorta

Temporal (Giant-Cell) Arteritis Most are >50 years of age (average 72) Women>men Females 70% Gradual onset 64% Prevalence high in Scandinavian countries VERY rare in Blacks and Hispanics

Fever/wasting syndrome Fever and chills Can sometimes present with Fever of Unknown Origin (FUO) Anorexia, weight loss Night sweats Weakness Depression Pain & Stiffness: Around shoulders and hips (polymyalgia rheumatica) – 15%

Cranial Arteries – most common Headaches…….severe Scalp tenderness +/- thickened vessels Ischemic optic neuropathy Loss of vision-diplopia Jaw claudication in 50% Tongue claudication CNS ischemia Strokes

Large-vessel GCA/aortitis 10-15% Arm claudication…femoral is rare Pulselessness Raynaud’s phenomenon Aortic aneurysm Aortic insufficiency PMR Often lack cranial involvement

Scalp necrosis Tongue gangrene Cranial and peripheral neuropathies Rare, isolated organ involvement

Temporal (Giant-Cell) Arteritis Physical Examination Very tender over temples Swollen, rope like temporal artery Optic disc swelling due to ischemia

Temporal (Giant-Cell) Arteritis

Temporal (Giant-Cell) Arteritis Investigations Complete Blood Count (CBC) Normochromic, normocytic anemia Reactive thrombocytosis WBC is usually normal Erythrocyte Sedimentation Rate (ESR) Significantly elevated C-Reactive Protein (CRP)

Diagnosis Biopsy in Temporal arteritis Biopsy abnormal site 4-6 cm. if not obviously abnormal If strong suspicion and normal biopsy, then biopsy opposite side. Doppler guidance?

Temporal Artery Biopsy

Temporal Artery Biopsy Inflammation Multi-Nucleated Giant Cell

Three of the following five criteria must be met to diagnosis GCA. 1-Age greater than 50 years 2-new headaches 3-abnormal temporal artery 4-ESR≥50mm/h and 5- positive temporal artery biopsy results for vasculitis

Therapy Relative EMERGENCY as patient can lose vision Urgent temporal artery biopsy (get the tissue) to confirm the diagnosis Initiate high-dose corticosteroids (40-60 mg per day) Relief DRAMATIC Taper by 10% per 2 weeks Duration – 9-12 months Steorid sparing drugs- MTX 10 mg/wk - maybe Low dose ASA can be considered

Polymyalgia Rheumatica

Polymyalgia Rheumatica A clinical syndrome of the middle aged and elderly characterized by pain and stiffness in the neck, shoulder and pelvic girdles,often accompanied by constitutional symptoms. The clinical response to small doses of corticosteroids can be dramatic.

Polymyalgia Rheumatica: Clinical features The musculoskeletal symptoms are usually bilateral and symmetrical. Morning Stiffness is the predominant feature Muscular pain is often diffuse and is accentuated by movement; pain at night is common.

Polymyalgia Rheumatica: Clinical features Systemic features include low-grade fever,fatigue, weight loss and an elevated ESR. Corticosteroid treatment is usually required for at least 2 years. .

Increased ESR AND CRP NO pathognomonic test No myopathy

All of the following criteria must be met to diagnose PMR: 1- Age greater than 50 years 2-aching and stiffness for at least 1 month,affecting at least two of the three above mentioned areas(ie,shoulders,neck, and pelvic girdle) 3-morning stiffness lasting at least 1 hour 4-ESR>40 mm/h 5-exclusion of other diseases except GCA and 6-rapid response to prednisone(<20 mgd)

Treatment of PMR Prednisone 15 mg Slow taper over 12 to 18 months Possible mtx use as 2nd line agent Look for temporal arteritis Concept of benign outcome

TAKAYASU’S ARTERITIS

Takayasu’s arteritis Takayasu’s arteritis is a chronic inflammatory disorder of unknown etiology primarily affecting the aorta and its major branches. Occurs most commonly in females under 40 years of age.

Takayasu’s Arteritis Pathophysiology Giant cells are seen invading the media & adventitia of affected vessels Bw52 or DR12 Immunogenetics of TA are less well defined Exposure to an unknown antigen precipitates an uncontrolled,inflammatory immune response that primarily targets large vessels

Takayasu’s Arteritis Clinical Picture TA had a triphasic course: 1. Early systemic complaints 2. Followed by symptoms related to vascular inflammation 3. Finally sequelae of vascular stenosis

Systemic phase: malaise, fever, night sweats and fatigue. Occlusive phase: upper limb claudication, headaches, postural dizziness and visual disturbances. Reduced or absent upper limb pulses. Arterial bruits over the carotid, abdominal and subclavian vessels

80% of patients had bruits most in the carotid arteries Elevated blood pressure occurred in 33% Diminished or absent extremity pulse occur over half of the patients with TA Nervous system symptoms occurred , in over half experienced dizziness Visual disturbance affected 1/3 of the patients, always bilateral

Takayasu’s Arteritis Evaluation ESR > 20mm/hr Ultrasonography and MRI have been used Angiography is considered the diagnostic gold standard Long stenotic lesions were seen in 98% of patients. Aneurysms were seen in 27%

Diagnosis - 3 of 6 criteria 1. onset age < 40 years 2. Limb claudication 3. decreased brachial artery pulse 4.unequal arm BP(>10 mmHg) 5.subclavian and artic bruit 6.Angiographic evidence of narrowing or occlusion of aorta

Takayasu’s Arteritis Management Glucocorticoids are the mainstay of therapy Other immunosuppressive therapies are used for patients who fail to respond to steroid therapy Azathioprine and Methotrexate are frequently used & cyclophosphamide has been used with some

Beta-adrenergic blockers and angiotensin-converting enzyme (ACE) inhibitors can be used to treat hypertension Percutaneous coronary transluminal angioplasty (PCTA) successfully restores patency in as many as 80% of these patients Bypass surgery can be done.

Takayasu’s Arteritis Prognosis 5-10 years survival rates are in the range of 80-97% Myocardial infarction, stroke, cardiac failure, aneurismal rupture are causes of death

Medium-sized Vessels Polyarteritis Nodosa Kawasaki’s Disease

Polyarteritis Nodosa (PAN) Necrotizing arteritis of medium sized muscular arteries Pathology: “fibrinoid necrosis”

Hepatitis B Virus Association Usually occurs during the first 6 months after infection Usually positive for HBAgs and e antigen

Epidemiology of Polyarteritis Nodosa Age: 20-70 years-old No racial or ethnic predilection Incidence 2-4/1,000,000 annual incidence 70-80/1,000,000/ in regions which are endemic for Hepatitis B

PAN Vasculature involved Superior mesenteric artery Celiac and hepatic arteries Renal artery Muscular arteries of the extremities

PAN-Clinical Manifestations Constitutional symptoms Fatigue Weight loss Fever

PAN -Clinical Manifestations Gastrointestinal Abdominal pain-intestinal angina ( postprandial abd. pain) Abdominal catastrophes, shock secondary to aneurysmal rupture and resultant hemorrhage Shock secondary to sepsis from intestinal ischemia or infarction Hepatomegaly

PAN -Clinical Manifestations Kidney (40%) -renal and interlobar arteries, rarely arcuate and interlobular arteries Hypertension Renal Insufficiency Renal Vasculitis, Rarely proteinuria and hematuria -paucimmune GN Angiography; microaneurysms with in kidney or large, wedge shaped infarctions

PAN -Clinical Manifestations Peripheral Nervous System Mononeuritis multiplex (e.g. wrist drop,foot drop) Central Nervous System Encephalopathy and strokes Skin Nodules or ulcers Purpura Digital gangrene

PAN -Clinical Manifestations Heart Pericarditis Congestive heart failure Arrhythmias Myocardial infarction testicular

Diagnosis PAN Kidney histology (Sural) nerve histology Muscle histology Skin histology? Colonoscopy, fecal blood? Visceral angiogram ANCA mostly negative

Polyarteritis Nodosa (3/10 - ACR) 1. Weight loss 4 Kg 2. Hypertension 3. Renal failure 4. Livedo reticularis 5. Testicular pain 6. Myalgia/Arthralgia 7. Mono- or polyneuropathy 8. Hepatitis B infection 9. Arteriogram findings 10.Biopsy

Prognosis of Polyarteritis Nodosa Untreated: 13% 5-year survival Treated: >70% 5-year survival

Mononeuritis Multiplex

PAN-Treatment 5 yr survival untreated: 13% Disease onset Prednisone 1 mg/kg q d Oral cyclophosphamide 2 mg/kg q d Duration of treatment At least one year +HBV PAN Interferon-α Lamivudine

Kawasaki Disease An acute febrile eruptive disease occurring most commonly in infants and children under 5 years of age. Vasculitis, especially involving coronary arteries, is a serious complication.

Kawasaki Disease Clinical Features Fever of unknown etiology lasting 5 days or more. Bilateral conjunctival congestion. Dry and red lips, reddening of oral cavity.

Kawasaki Disease-Clinical Features Acute nonpurulent swelling of the cervical lymph nodes. Polymorphous exanthema of the trunk without vesicles or crusts. Red palms and soles.

Bleeding and crust formation on the lips and cervical lymphadenopathy in Kawasaki disease.

Polymorphous exanthema on the limbs and trunk of an infant with Kawasaki disease.

Membranous desquamation of the fingertips.

Kawasaki Disease-Clinical Features Coronary arteries become affected in up to one - quarter of untreated patients; this can lead to myocardial ischaemia and infarction.

Coronary angiography. Left coronary artery aneurysm.

Treatment and Management recommended treatment for KD patients is a single, intravenously administered dose of immunoglobulin (IVIG, 2 g/kg) in conjunction with high-dose aspirin (80-100 mg/kg/day). The aspirin is continued until the ESR and platelet count have returned to the normal range. IVIG resistance -have included a second infusion of IVIG, a single infusion of infliximab, corticosteroids, and plasmapheresis.

SMALL VESSELS VASCULTITIS

Small Blood Vessels ANCA Related Immune Complex Related Miscellaneous Wegener’s, MPA, Churg-Strauss Immune Complex Related Hypersensitivity Vasculitis Cryoglobulinemic Vasculitis Connective Tissue Diseases Henoch Schonlein Purpura (IgA) Miscellaneous Malignancy Behcet’s Disease Inflammatory Bowel Disease

ANCA Related vasculitis

Anti-Neutrophil Cytoplasmic Antibody (ANCA) A collection of antibodies directed against components of granules inside the neutrophil Detected in the laboratory by Immunofluorescence Assay and by ELISA methods for specific antibodies

Anti-Neutrophil Cytoplasmic Antibody Immunofluorescence 2 patterns possible Cytoplasmic Perinuclear

Anti-Neutrophil Cytoplasmic Antibody Immunofluorescence - Disease Associations Wegener’s Granulomatosis c-ANCA = 75-80%, p-ANCA = 10-15%, Negative = 5-10% Microscopic Polyangiitis (MPA) c-ANCA = 25-35%, p-ANCA = 50-60%, Churg Strauss Syndrome (CSS) c-ANCA = 25-30%, p-ANCA = 25-30% Negative = 40-50%

Anti-Neutrophil Cytoplasmic Antibody Immunofluorescence & Antibodies c-ANCAs Anti-Proteinase 3 (PR3) p-ANCA Anti-Myeloperoxidase (MPO)

Wegener’s Granulomatosis (Granulomatous polyangitis)(GPA) Necrotizing vasculitis of arterioles,capillaries, and postcapillary venules Associated with anti-neutrophil cytoplasmic antibodies (ANCA)

Granuloma Nodular aggregate of macrophages or cells derived from the monocyte-lineage, which is typically surrounded by a “rim” of lymphocytes, and commonly associated with the presence of multinucleated giant-cells

GPA -Vasculature involved Upper respiratory tract arterioles and capillaries Lung arterioles and capillaries Pulmonary “capillaritis” Kidney Glomerulonephritis (“pauci immune”) Skin Peripheral Nervous system

Epidemiology of Wegener’s Granulomatosis Age: 25-60 years-old No racial or ethnic predilection Prevalence: 5-7/100,000

NOSE, SINUSIS, AND EARS 90% of patients have nasal involvement, often as the first manifestation of disease. symptoms include persistant rhinorrhea, unusually severe nasal obstruction, epistaxis, and bloody or brown nasal crusts.

NOSE, SINUSIS, AND EARS Cartilaginous inflammation lead to perforation of the nasal septum and collapse of the nasal bridge (a ‘’saddle-nose’’ deformity). Bony erosions of the sinus cavities are characteristic but only develop after long standing disease Both conductive and sensorineural hearing loss

“Saddle-nose” deformity resulting from collapse of the nasal cartilage

Saddle Nose Deformity

EYES Present with a variety of inflamatory lesions of the eye. Orbital pseudotumors behind the eye may lead to proptosis and visual loss through ischemia of the optic nerve. Scleritis causes photophobia and pain, scleral erythema.

Computed tomography scan of a patient with a left orbital mass. This was causing proptosis and visual loss through compression of the optic nerve.

Exophthalmos (patient's right eye). Enophthalmos (patient's left eye), both resulting from orbital pseudotumors

EYES Necrotizing scleritis may lead to scleral thinning, scleromalacia perforans , and visual loss. Peripheral keratitis lead to the syndrome of ‘’corneal melt’’. Episcleritis and conjunctivitis less serious ocular complications, they are very common

MOUTH Painful tongue ulcers occur An intense gingivitis known as “strawberry gums” is also characteristic of WG.

TRACHEA subglottic stenosis, tracheal inflammation and scarring below vocal cords, a potentially disabling manifestation largely specific to WG (relapsing polychondritis can also cause this lesion).

Subglottic stenosis. A web of scar tissue is evident just below the vocal chords, leading to narrowing of the subglottic area and inspiratory stridor.

LUNGS approximately 80% of patients have pulmonary lesions during the course of their disease. pulmonary symptoms include cough, hemoptysis, dyspnea, and sometimes pleuritic chest pain. Lung lesions are often asymptomatic may be detected only chest imaging is performed.

LUNGS radiolographic findings are pulmonary infiltrates and nodules. The infiltrates, which may wax and wane, are often misdiagnosed initially pneumonia. Nodules are usually multiple and bilateral and often cavitary. Pulmonary capillaritis may lead to hemoptysis and rapidly changing pulmonary infiltrates.

LUNGS Pulmonary capillaritis may lead to alveolar hemorrhage, hemoptysis, and rapidly changing alveolar infiltrates Diffuse alveolar hemorrhage is an immediately life-threatening complication. Large airway disease leading to a cobblestone appearance of the mucosal surface and bronchial narrowing (similar to subglottic stenosis) sometimes leading to postobstructive pneumonia.

Multiple bilateral pulmonary nodules can be seen, many of which have cavitated

Alveolar hemorrhage in a patient with Wegener granulomatosis. This has resulted in rapidly changing pulmonary infiltrates. There is also a nodular lesion in the right lung.

Pulmonary Nodules

KIDNEYS renal involvement is present in approximately 20% of patients at the time of diagnosis, develops in nearly 80% of patients at some point during the course of the disease. the clinical presentation of renal disease is rapidly progressive glomerulonephritis

KIDNEYS rapidly progressive glomerulonephritis: -- hematuria, red blood cell casts, proteinuria (usually non-nephrotic), and rising serum creatinine. Without appropriate therapy, loss of renal function may ensue within days or weeks.

OTHER ORGANS Constitutional symptoms - fever and weight loss, common in WG, serve as important indicators of an active inflammatory process. Nonspecific arthralgias and frank arthritis often occur early in the course of WG. arthritis of WG is migratory in nature may assume a variety of joint patterns, from a pauciarticular syndrome of lower extremity joints to a polyarthritis of the small joints of the hands.

OTHER ORGANS involvement of brain paranchyma has been reported, meningeal inflammation - more typical presenting as excruciating headaches and cranial neuropathies Mononeuritis multiplex may also accompany WG but is less characteristic of this disease than others (eg, polyarteritis nodosa, microscopic polyangiitis, and the Churg- strauss syndrome)

Mononeuritis multiplex. Wasting of the interosseous muscle between the thumb and first finger, caused by peripheral nerve infarction. (b) A patient with weakness bilaterally of foot dorsiflexion (“foot drop”), left greater than right.

OTHER ORGANS neuroendocrine complications - panhypopituitarism and diabetes insipidus WG rarely affects the heart, gastrointestinal tract, parotid gland, pulmonary artery, breast, or genitourinary organs.

ANCA associated > 90% have elevated titers of antineutrophil cytoplasmic antibodies

Anti-Neutrophil Cytoplasmic Ab (ANCA) Cytoplasmic reactivity (C-ANCA) Antigenic target = Proteinase 3 Assay: Anti-proteinase 3 Ab titers (ELISA)

Wegener’s Granulomatous (2/4 - ACR) Nasal or oral inflammation, Abnormal chest film Positive urinary sediment – RBC’s or RBC casts Biopsy -- necrotizing granulomatous vasculitis ANCA – Ab to proteinase 3, myelloperoxidase

Morbidity of Wegener’s Granulomatosis Permanent renal insufficiency- 42% End-stage renal disease- 11% Hearing loss- 35% Nasal deformities- 28%

Mortality of Wegener’s Granulomatosis Untreated: 10% survival at 2 years Treated: 80% survival at 10 years

Palpable Purpura

Treatment Regimen Prednisone 0.5-1 mg/kg q d (tapered) plus cyclophosphamide 2 mg/kg q d for approximately one year 85-90% response rate 75% complete remission 30-50% at least one relapse

Churg-Strauss syndrome (CSS) is a primary, multisystem, eosinophilic vasculitis associated with upper and lower respiratory tract disease and antineutrophil cytoplasmic antibodies (ANCAs). Clinical features include asthma, rhinitis, and sinusitis, with eosinophilia progressing to systemic vasculitis with cutaneous, cardiac, neurologic, gastrointestinal, and renal disease.

Churg-Strauss syndrome (CSS) characterized by respiratory tract and systemic vasculitis, eosinophilia, and extravascular granulomas. Also termed allergic granulomatosis and angiitis The mean age at diagnosis is 55 years with an equal sex incidence.

CSS-Histology Eosinophil infiltrates, extravascular granulomas, and vasculitis of small and medium vessels are characteristic features of CSS. Arteries, arterioles, venules, and veins may be involved in both the pulmonary and systemic circulations, and vasculitis can occur without granulomas. The pulmonary features combine focal necrotizing vasculitis with features of eosinophilic pneumonia.

Clinical Features three phases of CSS: an allergic prodromal phase, an eosinophilic phase, and a final vasculitic phase. The prodrome may last many years and comprises asthma, nasal allergy and polyposis, or other allergic manifestations. The majority of CSS patients have a history of allergy

Clinical Features The eosinophilic phase ;comprises a circulating eosinophilia and eosinophilic manifestations such as eosinophilic pneumonia, endomyocarditis, or eosinophilic gastroenteritis. This phase may fluctuate without therapy; after a variable period, features of systemic vasculitis emerge, including constitutional disturbance with fevers, weight loss, polyarthralgia, and polymyalgia.

Pulmonary disease asthma is a diagnostic criterion, Other pulmonary manifestations are infiltrates, caused by localized granulomatous vasculitis, pulmonary hemorrhage, pleural effusions, and the pulmonary consequences of cardiac disease. Chest radiography and computed tomographic assessment reveals parenchymal infiltrates in 75%.

Pulmonary disease Other abnormalities include small nodules, ground-glass opacities, bronchial wall thickening, and consolidation. Pulmonary manifestations respond to glucocorticoid and immunosuppressive therapy with reduction of asthma and resolution of radiologic changes

Ear, nose, and throat involvement symptoms of “allergic” rhinitis, nasal obstruction by polyps, deafness due to otitis media, and sinusitis often precede the diagnosis by several years. Sensorineural deafness either caused by inner ear inflammation or vasculitis of the auditory nerve Laryngeal involvement has been reported.

Cardiac involvement Cardiac involvement occurs in 50%, more frequently than with other vasculitides, is the major vasculitic cause of early death. Endomyocarditis leads to a cardiomyopathy and myocardial infarction both supraventricular and ventricular dysrhythmias occur and can result in sudden death. Pericardial effusion.

Renal disease The kidney is affected in 27% of patients hematuria with proteinuria, and a pauci-immune crescentic, necrotizing glomerulonephritis are the same as for the other ANCA-associated vasculitides.

Gastrointestinal involvement Intestinal involvement causes abdominal pain and can lead to perforation and peritonitis is an adverse prognostic factor for survival. Neurologic involvement Peripheral neuropathy occurs in over 50% of CSS patients, more frequent than in the other ANCA-associated vasculitides. an asymmetric mononeuritis multiplex Onset is usually with sudden motor deficit and can be painful.

Cutaneous features Purpura, reflecting small vessel vasculitis A specific lesion for CSS is the subcutaneous nodule. livedo reticularis, Digital gangrene of both the hands and feet has been seen

Treatment Subgrouping for treatment is done according to prognosis: prednisolone alone or with methotrexate or azathioprine is used for those without poor prognostic factors, with prednisolone with cyclophosphamide for those with poor prognostic factors. Relapse occurs in 75%. Intravenous methylprednisolone, intravenous immunoglobulin, interferon alfa, or rituximab is used for refractory or relapsing disease.

Microscopic polyangiitis Systemic necrotizing vasculitis Small-sized vessels Focal segmental necrotizing GN P-anca association NO granuloma Renal….RPGN

Microscopic polyangiitis vs PAN LUNG INVOLVEMENT BOTH HAVE MONONEURITIS Aneurysms in PAN Veins may be involved in MP

Treatment of MP Frequent relapses More prolonged treatment Prednisone AND cytoxan

Cutaneous PAN Chronic relapsing arteritis More in women 3 classes…. Mild…nodular ,livedo Livedo more prominent and ulcers Necrotizing livedo and acral gangrene • RX=PRED+/- “SUPPRESSIVES”

Vasculitis associated with CTD’s RA…..LCV in 10 -15% and rarely medium –vessel involvement SLE Sjogrens …usually LCV Scleroderma = CASE REPORT

Henoch Schonlein Purpura Age: 5-7 years old (range: 5-15) Children: 20/100,000 50% preceded by upper respiratory tract infection Adults: <1/100,000 Gender: male/female : 1.8/1

Vasculature involved Gastrointestinal tract Submucosal arterioles/venules Kidney Glomerulonephritis(mesangial) Skin Dermal arterioles, capillaries, and postcapillary venules

Clinical Manifestations Abdominal pain (“purpura” of the small bowel, i.e., submucosal hemorrhage) Intussusception Hematuria/proteinuria Renal insufficiency infrequent Purpura Arthralgia/arthritis

Pathogenesis Activation of the mucosal humoral immune compartment resulting in tissue (vascular) deposition of IgAcontaining immune complexes

Purpura of the Buttocks

IgA Deposition in the Mesangium

Prognosis of Henoch Schonlein Purpura 90-95% of patients exhibit spontaneous remission after 3-4 weeks, with 20-30% experiencing short-term relapses within the following 6-12 months

Treatment Supportive Hydration Bed rest Analgesia Non-steroidal antiinflammatory Agents

Conclusion Vasculitis 1 –ENT (Wegener‘s, Churg Strauss) – Lungs (Wegener‘s, Micro-PAN, Churg Strauss) –Kidneys (all) –Peripheral nerves (PAN, Churg Strauss) –GI (Henoch-Schönlein, PAN, Churg Strauss) – Skin (all)

Conclusion Vasculitis 2 Think of Arteritis, if – old, PMR, headache, fever (GCA) – young and large vessel disease (TA) • Joint involvement always possible • ANCA will help in a subset • Wherever possible go for histology