PPOK (Penyakit Paru Obstruksi Kronis)

Definisi
GOLD (Global Initiative for chronic obstructive lung disease): COPD adalah suatu penyakit yang ditandai dengan adanya hambatan aliran napas

yang tidak sepenuhnya refersibel. Hambatan

saluran napas tersebut biasanya bersifat progresif dan berhubungan dengan respon inflamasi abnormal paru terhadap partikel atau gas iritan.

Penyebab pembatasan saluran napas COPD

Irreversible:

-fibrosis dan penyempitan saluran napas

-hilangnya elastisitas oleh karena alfeoli telah rusak

Reversible
-akumulasi sel-sel inflamasi, mukus, plasma bronkus -kontraksi otot polos sentral maupun perifer -hiperinflasi dinamis selama exercise eksudat pada

Faktor Resiko
1. 2. Host Faktor Genes (alpha1-antitrypsin deficiency) Hyperesponsive Lung growth (prematur) Exposure Asap rokok (90%) Debu dan zat kimia di tempat kerja Infeksi (streptokokus, S. pneumonia, H. influenza) Status sosioekonomi

Yang termasuk dalam COPD

1. Bronchitis cronis 2. Emphisema 3. Bronchitis cronis dan emphisema

Bronchitis Cronis
Keadaan dengan produksi mukus trakeobronkial yang berlebihan sehingga menimbulkan batuk kronis berdahak minimal 3 bulan dalam setahun, sekurang-kurangnya 2 tahun berturut-turut, tidak disebabkan penyakit lainnya. Obstruksi saluran napas adalah persistent dan irreversibel.

etiology
Penyebab utama bronkitis kronis adalah asap rokok (90% kasus), infeksi saluran napas yang berulang, faktor genetik, inhalation chemical irritants.

Tanda dan gejala

1. Tanda utama bronkitis chronik adalah batuk persisten dengan produktif sputum 2. Batuk dan sputum meningkat pada pagi hari 3. Dyspneu pada saat aktivitas 4. Wheezing 5. Tahap akhir: cor pulmonal/heart fail edema peripheral Distensi vena leher

Pathogenesis
Faktor inisiasi (asap rokok 90%) Kerusakan bronkus, bronkiolus Hipersekresi mukus bronkospasme infeksi Obstruksi reversibel pada bronkus, bronkiolus Continued and repeat injury (smoking) Continued and repeated infection

Chronic inflamasi, Fibrosis mucous membrane, hyperplasi bronchial mucus gland and goblet cell, increase mucus production , increase bronchial wall thickness, increase cilliary dysfunction Chronic bronchitis

diagnosis
A. History 1. Lifestyle 2. Weight 3. Onset symptom 4. Sputum 5. Cough : smoker : overweight : after age 40 yr : excessive, purulen : chronic, more severe in morning : mild to moderate

6. Dyspneu

B. Keluhan pasien
1. 2. 3. 4. 5. 6. 7. Batuk kronis dengan seputum mukopurulen Malaise Nyeri otot Fatigue Insomnia Loss of libido Dyspneu (mild-moderate)

C. Tanda fisik
1. 2. 3. 4. 5. Edema : present Cyanosis : present in advanced disease Use of accesory muscle to breath: absent until end stage Antero posterior chest diameter : normal Auscultation of chest : wheezing, crackles, ronchi, depend on the severity of disease 6. Percusion : normal 7. Jugular vein distention : present 8. Other : evidence of right-sided heart failure ( cor pulmonal)

D. General diagnosis test

1. Chest radiografi: increase bronchial vasculer marking, enlarge horizontal heart 2. Artery blood gas analisys: decrease PaO2 ( <65 mm HG, increase Pa CO2) 3. ECG : Right axis deviation, right ventriculer hipertrofi, atrial arytmia 4. Hematocrit: polycitemia

Radiology

Pasien

E. Pulmonary finction test

Residual folume : increase

Total lung capacity : normal Forced expiratory volume: decrease Vital capacity : normal or slight decrease

right-sided heart failure ( cor pulmonal)

Cronic broncitis

Small pulmonary artery related to inflammation in the bronchial wall and the compensatory spasm of pulmonary blood vessel from hipoxia,

Produce pulmonary hypertension

Right ventricular end diastolic pressure increase

Leading to right ventricular dilation and right sided heart failure

Manifestasi klinik
Terjadi kelebihan cairan tubuh (edema) Jugular vena distensi Cyanosis ( stadium akhir)

Emphisema
Emphisema adalah kelainan pada paru yang ditandai dengan pembesaran abnormal yang permanen pada rongga udara distal bronkial terminal, disertai dengan kerusakan dinding alveolus , tanpa adanya fibrosis

Etiology
Cigarette smoking (>70 pack-year) Air polution Occupation ( working with or near asbeston) A1-antytripsin

 Emphisema cenderung berkembang dalam waktu

yang lama, dan sering terjadi pada seseorang dg

usia >50 th

Merokok dg jumlah >70 bungkus / tahun

merupakan faktor resiko tinggi terjadi PPOK

 proses penuaan normal, mulai sekitar usia 30,,

mencerminkan perubahan serupa dengan

yang terlihat pada emfisema :

Loss of alveoli Increase in the size of alveolar duct Loss of gas exchanging surface area (4% per decade), and decrease bronchial muscle

 Ketika emphisema terjadi pada usia mudadewasa, atau usia <50 th pada perokok Deficiency A1-antitrypsin
Penyakit herediter yang ditandai dengan penurunan jumlah A1 antitrypsin pada serum (2550 mg/dl)

<2% pasien emphisema

 A1-antitrypsin adalah suatu enzim yang berfungsi sebagai pelindung jaringan alveolus dari kerusakan yang disebabkan oleh zat proteolisis (protease) dari ( neutrophil, macrofagh ).

Type emphisema
1. Centriacinar ( centrilobuler emphisema) Dilatasi pada respiratory bronkhiolus Terjadi pada perokok berat dan berhubungan dengan bronkitis kronis 2. Panacinar (Panlobuler) emphisema Dilatasi pada bagian peripheral ( duktus alveolus dan alveolus) Berhubungan dengan A1 antitripsin deficiency 3. Paraseptal emphisema ( dilatasi distal acinus) 4. Irreguler emphisema (irreguler pd acinus karena terbentuk scar)

Panacinar emphisema

Centriasinar emphisema

patogenesis

Destruction of alveolar tissue

and septa Increased mucus secretion

Inflammation in the
bronchiolus Impaired in air way clearence Loss of radial traction with collapse of bronchial leading to

air trapping

Manifestasi klinis
1. 2. 3. 4. 5. 6. 7. Dyspneu is usually the first symptom Weight loss Barrel chest Prolong expiration Sits forward in a hunched-over position Breathes trough purs lips Pink puffer

Pink puffer

Barrel chest

diagnosis
A.History 1. Lifestyle : smoker 2. Weight : weight loss 3. Onset symptom : after age 50 yr 4. Sputum : mild, mucoid 5. Cough : minimal or absent 6. Dyspneu : progresif exertion dyspneu

Patient complain
Dyspneu on exertion, fatigue (kelelahan) , insomnia

C. Tanda fisik
1. Edema 2. Cyanosis : absent : absent

3. Use of accesory muscle to breath: present

4. Antero posterior chest diameter : barrel chest

5. Auscultation of chest
heart sound, prolong expiration

: decrease breath sound,

6. Percusion
7. Jugular vein distention 8. Other

: hyperersonance
: absent : purs lips breathing

D. General diagnosis test

1. Chest radiografi: hyperinflation, flat, low diafragma, normal or small vertical heart 2. Artery blood gas analisys: decrease PaO2 ( 60-80 mm HG), normal or increase Pa CO2 (increase with advancing disease) 3. ECG : normal or tall symmetrical P wave, tachycardi if hypocix 4. Hematocrit: normal

E. Pulmonary finction test

Residual folume : increase

Total lung capacity : increase Forced expiratory volume: decrease Vital capacity : decrease

Derajat PPOK
1. Pasien beresiko chronic symptom : batuk, sputum/dyspneu Exsposure to risk factor: merokok, polusi Normal spirometry: FEV1/FVC pasca bronchodilator >70% FEV1 80%

2. PPOK RINGAN FEV1/FVC < 70% FEV1 80% WITH OR WITHOUT SYMPTOMS 3. PPOK SEDANG FEV1/FVC < 70% 50%FEV1<80% WITH OR WITHOUT SYMPTOMS

4. PPOK BERAT FEV1/FVC < 70% 30%FEV1<50% WITH OR WITHOUT SYMPTOMS

5. PPOK SANGAT BERAT FEV1/FVC < 70% FEV1<30% OR FEV1<50% Predicted plus chronic respiratory failure

DIAGNOSA BANDING
1. Asma bronchial 2. Bronkiektasis 3. Tuberculosis 4. CHF (Congestive Heart Failure )

1. Asma bronchial
Adalah penyakit inflamasi kronis pada saluran nafas yang menyebabkan gejala berulang yi: wheezing, sesak nafas, dada terasa sesak, dan batuk, terutama pada malam hari, atau pada pagi hari.

 Gejala

tersebut

berhubungan dan airflow nafas) yg

dengan limitation bersifat

bronchokontriksi (pembatasan

saluran

reversibel (spontan atau dg treatment)

Type asthma
1. Atopic asthma

Begin in childhood
A positive family history

Asthmatic attack are often preceded by

allergy rinitis, urticaria

Trigger by environmental antigent : dust,

pollen, food.

allergen macrophage CD4 sell TH2 cell

pathogenesis

B sell
eosinophil

IGE
mediator

Mast sell
mediator neutrophil Monosit Limphosit Basofil

Bronchospasme Increase vascular permiability Mucus production Immediate phase (minutes)

Damage ephitelium

Late phase ( 4-8 hours)

2. Nonatopic asthma
Trigger by respiratory tract infection Virus ( Rhinovirus, parainfluenza Virus), bacteri. A positive family history is uncommon Serum IGE normal No other associated allergies Skin test are negative

 3. drug induce asthma

aspirin-inhibiting COX pathway of asam arachidonat-without affecting

lipoxygenase route- leukotrinbronchokontriksi

Tanda Klinis
sesak nafas, wheezing, tachicardi, tachypneu dada terasa sesak, Batuk dan peningkatan sputum ( thick/kental, scant/sedikit, sticky/lengket) ( beberapa pasien dg batuk kering dan yg lainnya dg batuk produktif.)

serangan asthma berlangsung hingga beberapa jam dan diikuti oleh batuk yang berkepanjangan.

diagnosis
1. Pemeriksaan fisik Sesak napas, wheezing, tachicardi, tacipneu, batuk. 2. Pemeriksaan sputum Charcot-leyden cristal (eosinophil membrane), eosinophil. 3. Spirometri Airflow obstruction is indicate by FEV1/FVC <75% 4. Pemeriksaan darah: peningkatan sell darah putih, eosinophil. 5. Radiographi Normal atau hyperinflasi dg diafragma mendatar pada progresif disease.

Bronchiektasis
Bronkiektasis adalah dilatasi dari bronchi Anak anak mempunyai resiko tinggi bronchiktasis karena faktor anatomi saluran nafas: small (kecil), soft (lunak,lemah), elastic bronchi. Bronchi pd anak anak sangat mudah mengalami kerusakan : overinflasi dan distensi karena inflamasi dan inveksi

patogenesis
Inflamasi dan infeksi berulang ( H. Influenza) Inflamasi menyebabkan kerusakan dinding central bronchi dan perifer bronchi dan bronchiolus = persistent dilation of the medium-size bronchi and bronchiolus Kerusakan ephitel cillia, squamous cell metaplasia, pembentukan pus, Menyebabkan obstruksi saluran nafas

Manifestasi klinik
Child ussualy present: 1. Chronic productive cough 2. Copious amount of purulent 3. Foul-smelling 4. Green or yellow sputum 5. Other clinic feature: hemoptysis, ronchi, bad breath, skin pallor.

Diagnosis
1. History of chronic productive cough 2. Produce copious amounts of foul-smelling, purulen sputum 3. Radiografi: increase bronchial marking, thickening of bronchial walls 4. Pulmonary function test: decrease airflow and vital capacyti in advanced cases 5. Arteri blood gas: hypoxemia (decrease PaO2), hypercapnea (increase PaCO2).

CHF (Congestive Heart Failure )

Penyebab utama CHF adalah ischemic cardiomiopathy dan hypertensi Manifestasi klinis, CHF dibagi 2: Left-sided heart failure

Rigth-sided heart failure

Left-sided heart failure

Disebabkan oleh: 1. Left ventrikel infarction 2. Cardiomiopaty 3. Aortic 4. Systemic hypertension

patofisiology
Left Ventricular Failure Backward effect Decrease ejection fraction Increase left ventricel preload Increasi left atrium pressure Increase pulmonary pressure Decrease tissue perfusion Forward effect

Decrease cardiac output

RAS activation Fluid retention

Increase right ventriculer after load

Pulmonary congestion

Right ventricular hypertrophi

Backward effect 1.Dyspneu on exertion 2.Orthopneu 3.Cough 4.Paroxysmal nocturnal dyspneu 5.Cyanosis 6.Basilar crackles

Forward effect 1. Fatigue (kelelahan) 2. Oliguria 3. Increase heart rate 4. Restlessness (gelisah) 5. Confusion 6. anxiety

Right ventricular failure

Penyebab: 1. Infark ventrikel kanan 2. Penyakit paru 3. Semua penyebab gagal ventrikel kiri

Patogenesis
Right Ventricular Failure

Backward effect Decrease ejection fraction Increase right ventricel preload Increasi right atrium pressure Sistemic congestion

Forward effect

Decrease output to left ventricle

Decrease left ventricle Cardiac output

RAS actifation Fluid retention

Decrease tissue perfusion

hepar

splen

gastroin testinal

Ren

Lower extremitas

Backward effect 1.Hepatomegali 2.Ascites 3.Splenomegali 4.Anorexia 5.Subcutan edema 6.Vena jugular distensi

Forward effect 1. Fatigue 2. Oliguria 3. Increase heart rate 4. Restlessness (gelisah) 5. Confusion 6. anxiety

TBC (Tuberculosis)
Kuman penyebab : mycobakterium tb (bakteri aerob, bentuk batang, tahan asam). Cara penularan :

Kuman masuk secara inhalasi (<5mikrometer) pada saat pasien batuk, bersin, bicara.

pathogenesis
M.Tuberculosis Jaringan Paru

Fagosit alveolar macrofagh

dindingM. TBC ( arabinomanan) Menghambat aktifasi macrofagh Multiplikasi M.TBC dimacrofagh Ghon tubercle Sel T CD4 TNF

Melisiskan sel terinfeksi

Peningkatan macrofagh

Manifestasi klinis
Batuk kronis >14-21 hari ( batuk kering- berminggu minggu/ berbulan bulan Purulent sputum- batuk darah ) demam subfebris (demam influenza) Berkeringat pada malam hari malaise (anoreksia, BB turun, sakit kepala, nyeri otot) Sesak nafas ( penyakit yg sudah lanjut)

TB Suspects Sputum AFB Microscopy Two or three smears + Only one smear + Three smears -

Non anti TB antibiotic

X-ray and medical officers judgment Repeat AFB No improve improve

One or more smears +

All smears -

X-ray and medical officers judgment

No TB YES TB

Management of COPD
1. Farmakology A. Bronchodilator anticholinergic,2-adrenergic reseptor agonist, methylxanthin B. Cortichosteroid C. Antibiotic 2. Non-farmacology Reducing risk factors, pulmonary rehabilitation, oxygen terapy, 3. Surgical therapy

Bronchodilator
A. Inhaled Anticholinergics Effect: decrease bronchoconstriction and glandular mucus Short-acting inhaled anticholinergic -such as : ipratropium -maximum bronchodilation in 1-2H, maintained approximately 4H Long-acting inhaled anticholinergic -such as: tiotropium -result in prolonged bronchodilation for 24H or more -dose: once-daily dosing regiment

COMBIVENT GENERIC Ipratropium Ipratropium and Albuterol

Ipratropium

Open airways by relaxing tight muscles around them. Always inhaled. Available as metered-dose inhalers, dry powder inhaler, or as a liquid for nebulization. Most often used together with short-acting or long-acting beta2-agonists.

POSSIBLESIDEEFFECTS Coughing Dry mouth Nausea Headache

Anticholinergics Short-Acting and Long-Acting

B. Inhal 2-adrenergic reseptor agonist

Effect : induce bronchodilation Short-acting B2-adrenergic receptor agonis SABAs -such as: terbutalin&albuterol -as needed basis to relieve symptoms because a rapid onset of action. -provide effect bronchodilation 4-6H

Albuterol

Albuterol and Ipratropium

Open airways by relaxing tight muscles around them. Usually inhaled, although occasionally taken as tablets. Available as metered-dose inhaler, dry powder inhaler, or as a liquid for nebulization. Carry the inhaler with you wherever you go for quick relief from sudden shortness of breath.

POSSIBLESIDEEFFECTS Rapid heartbeat Nervousness Tremors and shakiness Nausea Dry mouth and throat Increased blood pressure Muscle weakness Decreased blood potassium level

Beta2-Agonists Short-Acting

 Long-acting 2-adrenergic receptor agonists (LABAs) - formoterol and salmeterol - maintenance therapies for the long-term prevention and reduction of COPD-related symptoms -effect >12H

Salmeterol

Formoterol

Open airways by relaxing tight muscles around them. Often dry powdered inhalers, although they are occasionally taken as tablets. The inhaled medicines are only taken twice a day. Not to be used for quick relief of shortness of breath. POSSIBLESIDEEFFECTS (Very uncommon) Racing heart Tremors (shaking) Nervousness (gelisah)

LONG-ACTING BETA2 AGONISTS

C. Methylxanthines.
such as : aminophylline and theophylline use of these agents is considered controversial because: third-line

treatment option after inhaled 2-agonists and anticholinergic agents

because wide range of dose-related toxic effect & its limited clinical efficacy.

Adverse events associated with methylxanthine treatment include

possible fatal atrial and ventricular arrhythmias, convulsions, headaches, insomnia, nausea and heartburn.

theophylline continues to be used in the treatment of COPD

primarily because of its low cost

Bronchodilator Combination Therapy

Combined treatment with a 2-adrenergic receptor agonist, an anticholinergic agent, and/or theophylline have been shown to provide

additional improvements in lung function and health status compared with

single-agent therapy for COPD. Results of a study conducted by van Noord et al.demonstrated that

combination therapy with formoterol plus tiotropium improved

pulmonary function, as assessed by several lung function parameters (e.g. FVC and FEV1), more than improvements achieved with either component

alone.

Stage III to stage IV COPD

Oral corticosteroids (OCS) and ICS are used as add-on therapy in very specific

situations in the management of stable COPD.

The 2006 GOLD guidelines recommend ICS therapy with agents such as beclomethasone dipropionate, budesonide, fluticasone propionate, mometasone

furoate and triamcinolone acetonide for the treatment of patients with advanced
COPD (FEV1<50% predicted) who experience repeated exacerbations.

PULMICORT budesonid

Fluticasone

Beclomethasone

Reduce inflammation and swelling of the airways. Because they are inhaled, they generally only affect your lung and airways, not your entire body.

Inhaled Steroids

influenza vaccination
The influenza vaccine has been shown to reduce serious illness for patients with COPD by as much as 50%. Pneumococcal vaccine is also recommended for patients

>65years old = to reduce pneumococcal pneumonia in

patients with COPD

Non-pharmacologic Therapy for COPD

A. Reducing Risk Factors Smoking cessation Reducing and eliminating occupational exposures to airborne pollutants Consistent and persistent patient education and counselling

B. Pulmonary Rehabilitation exercise training, nutritional counselling, and patient education Exercise training should include aerobic and resistance exercises to improve aerobic capacity and muscle strength

Oxygen Therapy
Oxygen therapy decrease pulmonary hypertension, increases exercise capacity and lung function, improves the mental and

emotional states of patients.

Surgical Options Lung volume reduction surgery (LVRS), Lung transplantation

Managing COPD Exacerbations

Patients who experience acute exacerbations

commonly present with increased breathlessness, wheezing, tightness in the chest, increased cough

sputum production, change in colour and tenacity of

sputum, and fever. associated with infections (S.Pneumonia, H.influenza) of the tracheobronchial tree and with air pollution.