Dr. Müge Bıçakçıgil Kalaycı

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

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

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

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

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

Physical Examination Very tender over temples Swollen, rope like temporal artery Optic disc swelling due to ischemia

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

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 2 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 TB infection was briefly considered

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 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

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

Clinical Manifestations Constitutional symptoms
Fatigue Weight loss Fever

Abdominal catastrophes,
Gastrointestinal Abdominal pain Abdominal catastrophes, shock secondary to aneurysmal rupture and resultant hemorrhage Shock secondary to sepsis from intestinal ischemia or infarction Hepatomegaly

Clinical Manifestations
Kidney Hypertension Renal Insufficiency Renal Vasculitis, paucimmune GN Peripheral Nervous System Mononeuritis multiplex (e.g. wrist drop,foot drop) Skin Nodules or ulcers Purpura Digital gangrene

Heart testicular 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

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.

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.

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.

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

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: years-old No racial or ethnic predilection Prevalence: 5-7/100,000

Clinical Manifestations
Upper Respiratory Tract Chronic Sinusitis Chronic Otitis Lower Respiratory Tract Pulmonary nodules Alveolar hemorrhage(hemoptysis)

Mononeuritis multiplex Skin
Kidney Glomerulonephritis(crescentic) Peripheral Nervous System Mononeuritis multiplex Skin Purpura

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

Saddle Nose Deformity

Pulmonary Nodules

Palpable Purpura

Treatment Regimen Prednisone 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

Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome)
Characterized by eosinophil-rich, granulomatous inflammation of the respiratory tract and necrotizing vasculitis of small- to medium-sized vessels, associated with asthma and eosinophilia. 50% of patients with EGPA have antineutrophil cytoplasmic antibodies (ANCAs), usually with a specificity for myeloperoxidase (MPO).

Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome)
EGPA is a rare disease—significantly less common than the other forms of ANCA-associated vasculitis The distribution of cases is roughly equal between men and women. In recent years, associations between the use of leukotriene antagonists and EGPA have been reported.

Symptoms and Signs three disease phases are often recognizable:
The prodrome phase is characterized by the presence of allergic disease (typically asthma or allergic rhinitis, nasal polyps). This phase often lasts for several years. During the eosinophilia/tissue infiltration phase, striking peripheral eosinophilia may occur. Tissue infiltration by eosinophils is observed in the heart, lung, gastrointestinal tract, and other tissues.

Symptoms and Signs In the third phase, vasculitis, systemic necrotizing vasculitis affects a wide range of organs, ranging from the heart and lungs to the peripheral nerves and skin Ear: conductive hearing loss. sensorineural hearing loss have also been reported.

Symptoms and Signs Lungs
More than 90% of patients with EGPA have histories of asthma. During the vasculitic phase, necrotizing vasculitis and granuloma may be evident Peripheral Nerves Mononeuritis multiplex occurs with a remarkable frequency in EGPA, with often devastating effects. Vasculitic neuropathy was in 77% of the patients.

Symptoms and Signs Heart
Cardiac involvement also occurs and is a common cause of death. Congestive heart failure is the most common cardiac manifestation, coronary arteritis and valvular abnormalities have also been reported. Joints Nonspecific arthralgias and frank arthritis often occur early in the course of EGPA

none of which is speSymptoms and Signs
Skin none of which is speSymptoms and Signs cific: Palpable purpura, papules, ulcers, and vesiculobullous lesions are common. Nodular skin lesions are usually “Churg-Strauss granuloma” (cutaneous extravascular necrotizing granuloma). These tend to occur on the extensor surfaces of the elbows and other pressure points. Skin biopsy specimens in EGPA reveal eosinophilic infiltration of blood vessel walls.

Symptoms and Signs Kidneys
EGPA is less likely to cause end-stage renal disease than are other forms of ANCA-associated vasculitis. Acute kidney injury may be caused by an eosinophil-mediated interstitial nephritis. When glomerulonephritis does occur, the histopathologic findings are often indistinguishable from those of other forms of pauci-immune vasculitis (eg, GPA and MPA)

Laboratory Findings Eosinophilia (before treatment) is a sine qua non of EGPA. Eosinophil counts may comprise as much as 60% of the total white blood cell count. Eosinophil counts are usually sensitive markers of disease flares Most patients with EGPA also have elevated serum IgE levels. Antibodies to either proteinase-3 or MPO may be found.

Imaging Studies Pulmonary infiltrates are evident in approximately one third of patients with EGPA. These lesions are usually migratory infiltrates that occur bilaterally. Pulmonary hemorrhage is unusual but has been reported.

Microscopic Polyangiitis (MPA)
is the most common cause of the pulmonary-renal syndrome of alveolar hemorrhage and glomerulonephritis. Antineutrophil cytoplasmic antibodies (ANCAs) are often critical in making the diagnosis, but a significant minority of patients are ANCA-negative. The majority of patients with MPA directed against myeloperoxidase (MPO-ANCA).

The term “polyangiitis” is preferred to “polyarteritis” for MPA because of the disease’s tendency to involve veins as well as arteries. involves necrotizing vasculitis with few or no immune deposits affects small blood vessels (capillaries, arterioles, or venules) and possibly medium-sized vessels; and demonstrates a tropism for the kidneys and lungs.

The male:female ratio is approximately 1:1. The typical patient is middle-aged to elderly, but the disease may affect people of all ages. The mean age at diagnosis for MPA patients is approximately 60 years

Symptoms and Signs MPA is classified appropriately as a “pulmonary-renal syndrome,” Constitutional: Weight loss, anorexia, fevers Head, Eyes, Ears, Nose, and Throat: Rhinitis, tongue or other oral ulcers; occasional purpuric lesions on palate; ocular inflammation (eg, sclerouveitis) reported but rare

Symptoms and Signs Gastrointestinal; Skin
The skin manifestations of MPA include all of the cutaneous lesions associated with small-vessel vasculitis palpable purpura, papules, vesiculobullous lesions, splinter hemorrhages Gastrointestinal; Mesenteric vasculitis with microaneurysms in some patients

Symptoms and Signs Lungs
The principal pulmonary manifestation of MPA is capillaritis, which leads to alveolar hemorrhage and often to hemoptysis. nonspecific infiltrates; pulmonary fibrosis; pleural effusions

Symptoms and Signs Kidneys:
Renal involvement is seen in at least 80% of patients with MPA. The classic presentation of renal disease in MPA is a rapidly progressive glomerulonephritis the pathologic features of renal disease in MPA are indistinguishable from other forms of pauci-immune glomerulonephritis—namely, a necrotizing, crescentic lesion

Renal involvement may present with urinary abnormalities such as proteinuria, microscopic hematuria, and red cell casts. Red blood cell casts

Symptoms and Signs Joints: Peripheral nerve :
Migratory pauciarthritis or polyarthritis or arthralgias; arthritis is nondestructive Peripheral nerve : Vasculitic neuropathy is a potentially devastating complication of MPA. The nerve involvement typically occurs in the pattern of a distal, asymmetric, axonal polyneuropathy (mononeuritis multiplex).

The first symptoms of vasculitic neuropathy are usually sensory, with numbness, tingling, and dysesthesias. Muscle weakness and wasting follow the infarction of motor nerves

Laboratory Findings Test Typical Result Complete blood cell count
Normochromic, normocytic anemia; acute, severe anemias possible in alveolar hemorrhage Mild to moderate leukocytosis common, usually not exceeding 18 × 109/L Moderate to pronounced thrombocytosis typical, ranging from platelet counts of 400 × 109/L to occasionally >1000 × 109/L Urinalysis with microscopy Hematuria (ranging from mild to so high that red blood cells are too numerous to count) Red blood cell casts Proteinuria (nephritic range proteinuria in a small minority) Erythrocyte sedimentation rate/C-reactive protein Dramatic elevations of acute phase reactants are typical, generally with good correlation to disease activity Rheumatoid factor Positive in 40–50% of patients, often leading to diagnostic confusion withRA ANCA Positive in 70% of patients with MPA (and probably a higher percentage of patients with generalized disease)

Special Tests Tissue Biopsy Nerve Conduction Studies
Renal ,Skin?, Lung ? Renal biopsy findings, not specific for MPA, İmmunofluorescence studies of renal biopsies in MPA confirm the “pauci-immune” nature of the renal involvement Nerve Conduction Studies Nerve conduction studies may reveal the characteristic asymmetric, axonal sensorimotor neuropathy. the sural nerve candidates for biopsy,

Serologic Testing for ANCA
Three fourths of MPA patients are ANCA-positive. In MPA, the classic pattern of serum reactivity upon immunofluorescence testing is perinuclear staining (P-ANCA). The P-ANCA pattern in MPA patients is usually caused by antibodies to MPO, a constituent of the primary granules of neutrophils

Treatment the combination of cyclophosphamide and glucocorticoids is the cornerstone of treatment . Rituximab has been demonstrated to be as effective as CPA in ANCA-associated vasculitis (AAV)

Comparison of the Features of MPA, GPA, and PAN.
Vessel size Small to medium Medium Vessel type Capillaries, venules, and arterioles; sometimes arteries and veins Muscular arteries Granulomatous inflammation No Yes Lung involvement Yes (pulmonary capillaritis) Yes (pulmonary nodules, often cavitary) Glomerulonephritis Renin-mediated hypertension ANCA-positive 75% 60–90% Hepatitis B association Yes (<10% of cases now) Microaneurysms Rarely Typically Mononeuritis complex Commonly (60%) Occasionally

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 % and rarely medium –vessel involvement SLE Sjogrens …usually LCV Scleroderma = CASE REPORT

Henoch-Schönlein Purpura (HSP)
HSP is nonthrombocytopenic purpura, caused by inflammation in blood vessels of the superficial dermis. The pathologic hallmarks of HSP are a leukocytoclastic vasculitis and deposition of immunoglobulin (Ig) A in the walls of involved blood vessels.

Henoch-Schönlein purpura
the most common form of systemic vasculitis in children, The peak incidence is in the first and second decades of life 90% of patients are younger than 10 years of age male to female ratio of 2:1. The incidence is significantly lower in adults, with a mean age at presentation of 50 years. HSP is more prevalent in the winter months. 50% preceded by upper respiratory tract infection

HSP Clinical Findings -Symptoms and Signs
The classic full presentation includes acute onset of fever, palpable purpura on the lower extremities and buttocks, abdominal pain, arthritis, and hematuria. All components of this presentation are not required for the diagnosis, In adults, the diagnosis should be confirmed by biopsy

Skin The cutaneous findings of HSP include purpura
usually palpable, although sometimes not urticarial papules, and plaques. Lesions are concentrated over the buttocks and lower extremities tend to involve the small blood vessels in the superficial dermis. Localized edematous swelling of the subcutaneous tissues of the lower extremities is frequently observed

Purpura of the Buttocks

Joints occurs in more than 80% of patients with HSP,
manifests itself as arthralgias or arthritis in large joints, especially the knees and ankles and, to a lesser degree, the wrists and elbows. migratory patterns of joint involvement are common. the arthritis is nondeforming in nature.

Gastrointestinal Tract
Approximately 60% of patients with HSP have abdominal pain and 33% have evidence of gastrointestinal bleeding. Abdominal symptoms result from edema of the bowel wall as well as hemorrhage induced by mesenteric vasculitis. Abdominal pain may precede the appearance of purpura by up to 2 weeks, leading often to diagnostic confusion and occasionally to invasive testing or even laparotomy. The abdominal pain is typically colicky and may worsen after eating (ie, intestinal angina).

Gastrointestinal Tract
upper or lower gastrointestinal bleeding. mesenteric ischemia in HSP rarely leads to gut perforation. Purpuric lesions may be seen on endoscopy, commonly in the descending duodenum, stomach, and colon. Gastrointestinal involvement in children with HSP can cause intussusception, HSP-associated intussusception is usually ileoileal.

Kidney Renal involvement is the most potentially debilitating complication of HSP. forty percent of patients with HSP have renal disease. renal involvement is more frequent and tends to be persistent in adults, adults have a higher risk of developing end-stage renal disease than children. glomerulonephritis almost always appears after the development of skin manifestations.

Kidney The clinical hallmark of nephritis in HSP is hematuria,
often macroscopic, but more typically microscopic. The hematuria can be transient, persistent, or recurrent. Proteinuria never occurs in the absence of hematuria in the acute setting. The most common renal lesion (60% of cases) is a focal, proliferative endocapillary glomerulonephritis. Crescents are present in up to 40% of biopsies. Direct immunofluorescence studies characteristically demonstrate IgA deposition in the mesangium.

IgA Deposition in the Mesangium

Other Organs Pulmonary and central nervous system (CNS) complications of HSP have been described, but these are very rare. usual lung manifestation of the disease is alveolar hemorrhage. Seizures are the usual CNS manifestation of HSP Testicular involvement occurs in up to 10% of boys with this disease and may mimic torsion.

Modestly elevated acute phase reactants may be observed.
Laboratory Findings Modestly elevated acute phase reactants may be observed. Urinalysis with microscopy: Hematuria (ranging from mild to too numerous to count red blood cells). Red blood cell casts. Proteinuria (nephrotic range proteinuria in a small minority). Sixty percent of patients have an elevated serum IgA.

Treatment Supportive Hydration Bed rest Analgesia
Nonsteroidal anti-inflammatory drugs may alleviate arthralgias but can aggravate gastrointestinal symptoms and should be avoided in any patient with renal disease.

Treatment Dapsone (100 mg/d) may be effective in cases of HSP,
perhaps through interference with the interactions of IgA and neutrophils. high-dose methylprednisolone followed by oral prednisone or high-dose prednisone combined with azathioprine or cyclophosphamide may help patients with severe nephritis In most cases, HSP follows a self-limited course,resolves without substantial morbidity, and does not recur. The vast majority of cases resolve within 6–8 weeks.

Conclusion Vasculitis
–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)

Dr. Müge Bıçakçıgil Kalaycı

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