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Pulmonary vasculitis. Sanitra Sirithangkul M.D. Division of Pulmonary and Critical care Department of Pediatrics Phramongkutklao Hospital. Systemic diseases and the lung. • relatively rare in pediatric practice • CNT diseases : SLE, dermatomyositis • Pulmonary vasculitis

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pulmonary vasculitis
Pulmonary vasculitis

Sanitra Sirithangkul M.D.

Division of Pulmonary and Critical care

Department of Pediatrics

Phramongkutklao Hospital

systemic diseases and the lung
Systemic diseases and the lung

• relatively rare in pediatric practice

• CNT diseases : SLE, dermatomyositis

• Pulmonary vasculitis

• Inherited CNT disorders : Ehlers-Danlos

• Mucopolysaccharidoses, familial

dysautonomia, sarcoidosis

•LCH, pulmonary lymphangiomatosis

Pediatr Respir Rev 2005;6:181-189

systemic diseases and the lung1
Systemic diseases and the lung

• may cause significant respiratory

complications

• treatment : often requires corticosteroids &

immunosuppressants

• outcome : variable

• when they affect the lung, carry a small but

significant mortality

Pediatr Respir Rev 2005;6:181-189

systemic vasculitis
Systemic vasculitis

•true incidence in children is not known

• around 2-5 cases/million/year

• pathology of vasculitis : cellular

inflammation, vessel destruction,

tissue necrosis

• clinical features : site, size and type

of vessel involved

classification vasculitis
Classification ของในโรคในกลุ่ม Vasculitis

Primary idiopathic vasculitis

- Small vessel

• Wegener’s granulomatosis

• Microscopic polyangitis

• Churg-Strauss syndrome

• Idiopatic pauci-immune rapidly

progressive glomerulonephritis

• Isolated pauci-immune pulmonary

capillaritis

slide6

Primary idiopathic vasculitis

- Medium vessel

• Polyarteritis nodosa

• Kawasaki disease

- Large vessel

• Giant cell arteritis

• Takayasu’ s arteritis

Primary immune complex-mediated

vasculitis

• Goodpasture’s syndrome

• Henoch-Schonlein purpura

• Behcet’s disease

slide7

Classification ของในโรคในกลุ่ม

vasculitis (cont.)

Secondary vasculitis

- Classic autoimmune disease

• Systemic lupus erythematosus

• Rheumatoid arthritis

• Polymyositis / dermatomysitis

• Scleroderma

• Antiphospholipid antibody syndrome

slide8

Secondary vasculitis

• Essential cryoglobulinemia

• Inflammatory bowel disease

• Hypocomplementemic urticarial

vasculitis

• Drug-induced (e.g., propylthiouracil,

diphenylhydantoin)

• Paraneoplastic

• Infection

pulmonary vasculitis1
Pulmonary vasculitis

• one component of systemic vasculitis

• pathology : fibrin thrombi, fibrinoid

necrosis

• the inflammation may lead to

- a progressive destruction of pulmonary

circulation

- granuloma formation

- end – organ failure

vasculitis
โรคในกลุ่ม vasculitis ที่ทำให้เกิดพยาธิสภาพที่ปอด
  • Pulmonary involvement common
  • - Wegener’s granulomatosis*
  • - Goodpasture’s syndrome*
  • - Idiopathic pulmonary hemosiderosis
  • - Kawasaki disease
  • * Positive serum anti-neutrophil cytoplasmic antibody (ANCA)
slide11

โรคในกลุ่ม vasculitis ที่ทำให้ เกิดพยาธิสภาพที่ปอด (ต่อ)

B. Pulmonary involvement uncommon

- Henoch-Shonlein purpura

- Churg-Strauss vasculitis*

- Polyarteritis nodosa*

- Takayasu arteritis

- Temporal arteritis

- Serum sickness

- Cryoglobulinemia

* Positive serum anti-neutrophil cytoplasmic antibody (ANCA)

clinical scenarios suggestive of vasculitis
Clinical scenarios suggestive of vasculitis

1. Diffuse alveolar hemorrhage (DAH)

- hemoptysis

- diffuse alveolar infiltration in

CXR

- a drop in hematocrit

diffuse alveolar hemorrhage
สาเหตุของ Diffuse alveolar hemorrhage

With pathologic capillaritis

- Primary idiopathic small vessel

vasculitis

- Primary immune complex-mediated

vasculitis

- Secondary vasculitis

slide15

Without pathologic capillaritis (bland

hemorrhage)

- Idiopathic pulmonary hemosiderosis

- Coagulopathy

- Mitral stenosis

- Inhalation injury

- Goodpasture syndrome

- Systemic Lupus erythematosus

- Bone marrow transplantation (associated with diffuse alveolar damage)

- Drug – associated disease (e.g., chemotherapeutic agents)

clinical scenarios suggestive of vasculitis1
Clinical scenarios suggestive of vasculitis

2. Acute glomerulonephritis

- rapidly progressive

glomerulonephritis (RPGN)

- to be considered

SLE, post-infectious GN, IgA

nephropathy, MPGN,

ANCA-associated vasculitis

clinical scenarios suggestive of vasculitis2
Clinical scenarios suggestive of vasculitis

3. Pulmonary-renal syndrome

- DAH / pulmonary capillaritis +

glomerulonephritis

4. Destructive upper airway lesions

5. Chest imaging findings

6. Palpable purpura

7. Mononeuritis multiplex

8. Multisystem disease

specific testing
Specific testing

1. Antineutrophil cytoplasmic

antibodies (ANCA)

- circulating autoantibodies against

intracellular antigens found in neutrophils

- cytoplasmic ANCA (c-ANCA),

perinuclear ANCA (p-ANCA)

specific testing1
Specific testing

2. Radiographic imaging

- CT chest : cavity, nodule, diffuse

ground glass opacification

- CT sinus

3. Bronchoscopy

- assess for infection / alveolar

hemorrhage / endobronchial lesion

specific testing2
Specific testing

4. Diagnostic biopsy

- skin, sinus or upper airway lesions

- renal biopsy

- lung biopsy

- collect tissue in saline for culture

- frozen tissue for

immunofluorescence

- formaline-fixed tissue for H&E

wegener s granulomatosis wg
Wegener’s granulomatosis (WG)

• the most common of the ANCA-associated

vasculitis

• triad : upper airway disease, lower

respiratory tract disease,

glomerulonephritis

• abnormal CXR findings : alveolar,

interstitial, mixed infiltration

nodule/cavity

wegener s granulomatosis wg1
Wegener’s granulomatosis (WG)

• c-ANCA / antiPR3 positive 85-95% of

active, systemic WG

• poor outcomes : advanced age, severe

renal involvement, alveolar hemorrhage,

anti PR3 positive

microscopic polyangiitis mpa
Microscopic polyangiitis (MPA)

• long prodromal phase of constitutional

symptoms → development of RPGN

• pulmonary involvement seen in up to

30%

• most common pulmonary involvement :

DAH with pulmonary capillaritis

microscopic polyangiitis mpa1
Microscopic polyangiitis (MPA)

• p-ANCA +ve 50-70%, anti MPO +ve

35-65%, c-ANCA +ve 10-15%

• pathology : focal, segmental necrotizing

vasculitis, mixed inflammatory infiltrate

without granuloma

churg strauss syndrome css
Churg-Strauss syndrome (CSS)

• to be considered when other eosinophilic

lung diseases are in the differential or

• difficult-to-control asthmatic patients

develop significant cardiac, GI or

neurologic disease

• triad : asthma, hypereosinophilia,

necrotizing vasculitis

churg strauss syndrome css1
Churg-Strauss syndrome (CSS)

• pulmonary hemorrhage and

glomerulonephritis : less common

• p-ANCA /anti MPO +ve 50-75%,

c- NCA +ve 10%

• mortality & morbidity due to cardiac

complications, GI, status asthmaticus

& respiratory failure

therapy
Therapy

•induction of remission : 12 months

•maintenance : 12-18 months

- cyclophosphamide → azathioprine/

methotrexate

- additional agents : mycophenolate

mofetil (MMF), leflunomide, cyclosporine

- Pneumocystis carinii prophylaxis with

trimetroprim-sulfametoxazole

monitoring
Monitoring

•to minimize morbidity & mortality of the

vasculitides and their therapy

•differential diagnosisin pts with clinical

deterioration

- infection

- drug toxicity

- disease relapse

- a new unrelated problem

take home message
Take home message

•Pulmonary vasculitis is one

component of a variety of systemic

vasculitis

• Early diagnosis using common

clinical scenarios and appropriate

investigations

take home message1
Take home message

• Aggressive early treatment to

minimize disease related mortality &

irreversible damage

• Regular monitoring for disease

activity and medication toxicity