PENYAKIT

PARU
RESTRIKTIF

Pembimbing: dr. Tri Damiati, Sp.KFR

Penyakit paru restriktif…

– Penyakit dg keterbatasan ekspansi paru, yang diakibatkan karena kelainan pada

otot, parenkim, pleura, atau tulang
– In restrictive lung disease, the primary limitations are low tidal volumes from an
inability to expand the chest wall (extrinsic restriction) or from very
noncompliant lung tissue (intrinsic restriction)
– Restriksi  perubahan ventilasi paru dan distribusi respirasi gas cenderung
menyebabkan hiperventilasi pada tahap awal penyakit yang kemudian menjadi
hipoventilasi. Keduanya ini dapat menyebabkan gangguan keseimbangan asam-
basa.
In extrinsic restrictive disease the parenchyma of the lung
is normal and gas exchange is preserved, meaning that
treatment is usually respiratory muscle training and
mechanical ventilatory support as needed
– Intrinsic restrictive lung diseases there may be a profound
associated hypoxemia from severely decreased diffusion capacity
of scarred lung tissue.
– Patients with parenchymal restrictive disease classically have
severe hypoxemia and may need high-flow supplemental oxygen.
Patients with end-stage intrinsic restrictive disease can have
ventilatory failure with hypercarbia and hypoxemia and may be
refractory to ventilatory support, and lung transplantation is then
often the only remaining treatment option
– Restrictive pulmonary disease is most commonly caused by neuromuscular
disorders, thoracic injuries, such as spinal cord injury (SCI), scoliosis, or obesity.
– Injury to the cervical and upper thoracic spinal cord disrupts the function of
inspiratory and expiratory muscles, as reflected by the reduction in spirometry
and lung volume variables.
– According to statistics available from the National Spinal Cord Injury Statistical
Center, as of March 2014 there are an estimated 240,000 to 337,000 persons
with SCI or spinal cord dysfunction in the United States. Of these patients, 79%
are male, 14% have complete tetraplegia, and 45% have incomplete tetraplegia.
Duchenne muscular dystrophy is one of the more common neuromuscular
diseases that cause restrictive pulmonary dysfunction,
Patogenesis alveolar hipoventilasi
 penyakit paru restriktif
Mechanical load Chemical
(parenchymal/ske compesation
letal deformity)

Alveolar Pulmonary
Hypoxemia,
WoB TV hypoventilatio hypertension
hypercapnia
n

Muscle Cor
power pulmonale
…Penyakit paru restriktif

Gangguan paru restriktif  dpt dilihat dari tes fungsi paru :

– Penurunan FEV1
– % FVC : Normal / tinggi
 Rehabilitasi : memperbaiki kapasitas fungsional, mencegah infeksi paru dan
akibatnya yang merusak, serta memperlambat progresifitas
Penyebab Penyakit paru
restriktif
1. Gangguan muskuloskeletal : kyphoskoliosis, ankylosing spondilitis,
pectus excavatum

2. Gangguan neuromuskuler : SCI, poliomielitis, hemiplegia, muscular

dystrophy, GBS (infectious polyneuritis)

3. Penyakit parenkim : sarkoidosis, fibrosis, tbc, pneumoconiosis &

occupational lung disease : silicosis, black lung disease

4. Operasi eksisi & reseksi paru minimal - luas

5. Gangguan pada pleura : pleuritis, efusi pleura, pneumothorax

Kelainan restriksi karena
muskuloskeletal
– Kyphoskoliosis
– Ankylosing spondilitis
– Pectus excavatum
 Kifoskoliosis…

– Kelainan bentuk progresif yang ditandai dengan perubahan angulasi

lateral & posterior dari tulang belakang, yang menyebabkan gangguan
fungsi paru bertahap dan gangguan sekunder dari fungsi jantung.
– Penyebab bervariasi : kelainan neuromuskular, abnormalitas kongenital,
dan penyakit intrinsik spinal namun 70 – 80 % idiopatik.
– Kelainan bentuk yg progresif  mempengaruhi pengembangan rongga
thoraks dan abdomen, menyebabkan pengembangan asimetrik iga,
penonjolan dinding dada anterior dan sternum serta pengurangan
dimensi vertical thorax
…Kifoskoliosis

– Perubahan bentuk tulang  membatasi ekspansi thorax dan mobilitas

diafragma, secara mekanik menyempitkan (constriction) vascular bed paru yang
mengakibatkan gangguan ventilasi-perfusi.
– Paru-paru yang tertekan khususnya pada sisi cekung (concave)
– Gangguan fungsi respirasi bervariasi: ringan – berat.
– Kurva torakal < 65° jarang sampai mengganggu ventilasi
…Kifoskoliosis

– Impairment yang lebih besar  peningkatan WoB gradual  hipoventilasi

alveolar progresif yang menyebabkan hipoxemia & hierkapnia  hipertensi
pulmonal  cor pulmonale.
– Pasien jarang datang sebelum gejala jelas terasa, gejala yang mungkin muncul
antara lain: pernafasan cepat & dangkal, sesak saat latihan, pada kasus yang
lebih berat dapat terjadi sesak saat istirahat dan beberapa gejala insufisiensi
kardiopulmonar seperti sianosis, somnolen, gangguan jantung dan koma.
 …Kifoskoliosis

Pemeriksaan penunjang :
– X-rays : kelainan bentuk rongga thoraks dan abdomen
– EKG : hipertofi ventrikular kanan
– Analisa gas darah arteri: bisa menunjukkan hiperventilasi-hipoventilasi tergatung
stage
– Tes fungsi paru  penurunan volume paru (Inspiratory Capacity, Vital Capacity,
Expiratory Reserved Volume), Rasio Resdual Volume/Total Lung Capacity
cenderung meningkat terutama bila kurva spinal > 90°
…Kifoskoliosis

– Operasi  memperbaiki fungsi paru

– Brace  kadang malah mengganggu fungsi paru karena penekanan dinding
toraks dan abdomen
Ankylosing spondilitis…

– Penyakit inflamasi progresif yang melibatkan tulang belakang serta soft tissue
yang melingkupi nya.
– Sering tjd pd pria muda usia 30-an.
– Berhubungan dengan adanya HLA-B27 Ag.
– Pada pemeriksaan fisik dapat ditemukan: synovitis sacroiliac, sendi apophyseal
dan costovertebral.
– X-rays : Bamboo spine  osifikasi annulus fibrosus & pembentukan
syndesmophytes (bony bridges) + fibrosis  bony ankylosis
…Ankylosing spondilitis

– Rotasi aksial dari iga terhambat  gangguan mobilitas toraks & efektifitas kerja
otot toraks.
– Test fungsi paru : penurunan total lung capacity, residual volume dapat
normal/penurunan ringan
– Chest physiotherapy dini khususnya breathing exercise dapat memperlambat
progresifitas. Mobilisasi iga dengan latihan chest expansi juga dapat
memperlambat ankylosing
Pectus excavatum

– Malformasi kongenital toraks dengan penurunan bagian bawah sternum dan

perkembangan abnormal dari anterior diafragma yang mengakomodasi bentuk
konfigurasi sternum.
– Ketrebatasan volume paru tergantung dari luasnya deformitas.
– Kegagalan respirasi dapat terjadi pada deformitas yang berat.
– Pada kondisi serius diperlukan intervensi operasi dengan standar breathing
exercise pre & post operasi
Restriksi karena gangguan
neuromusmular
– Muskular distrofi
– ALS (Charcot’s disease)
– GBS (infectious polyneuritis)
– Hemiplegia
– Quadriplegia : SCI
Muskular distrofi…

– Miopati degeneratif primer yang diturunkan secara genetik.

– Mempunyai banyak tipe, secara fungsional dibedakan berdasarkan kelompok
otot dominan yang terkena, usia mulai terkena, kecepatan progresifitas distrofi.
– Diagnosis berdasarkan anamnesis dan pemeriksaan fisik, pemeriksaan genetic
dan patologis serta uji biokimia & elektrodiagnostik.
– Semua muskular distrofi akan mengganggu pernafasan
…Muskular distrofi

Permasalahan pada muscular distrofi

– Kelemahan otot2 trunk  scoliosis atau kifoskoliosis
– Kelemahan otot2 respirasi  kemampuan batuk terganggu
– Cardiac myopati juga dapat menjadi factor yang memperberat masalah respirasi
pada muscular distrofi
ALS (Charcot’s disease)

– Merupakan suatu miopati berat dalam hal penyebab gangguan paru restriktif
merujuk suatu miopati berat.
– Progresifitas penyakit ini sangat cepat sehingga penatalaksanaan dilakukan
secara intensif terus menerus
– Pada tahap akhir LTOT & IPPB dapat membantu
GBS (infectious polyneuritis)

– Penatalaksanaan biasanya sama dengan miopati lainnya, yang membedakannya

adalah prognosis GBS ini lebih baik
– Pada beberapa kasus dapat terjadi
Restriksi karena gangguan
ekstraparu
– Obesity-hypoventilation syndrome, asites, tumor abdomen, kista ovarium yg
besar, kehamilan trimester 3  restriksi ekspansi paru
Restriksi karena penyakit
parenkim

– Dibagi kedalam 2 tipe : infiltratif & granulomatosa/fibrotik.

– Onset tidak tergantung tipe, dapat akut dengan pneumonitis dan shock lung maupun kronis
seperti pada sarcoidosis, pneumoconiosis dan penyakit okupasional lainnya missal silicosis,
farmer’s lung.
– Pemeriksaan fisik biasanya bervariasi
– Moderate-severe exertional dyspnea gejala yang biasa ditemukan
– X-rays : patchy infiltrations, hilar nodes enlargement, honeycomb pattern.
– EKG : hipertrofi ventrikular kanan
– Test f/ paru : penurunan TLC, VC, RV.
– Th/ : Chest PT, KS th/ u/ sarcoidosis, penyakit kolagen vaskular, idiopatic-interstitial-alveolar
disease.
– Restrictive lung diseases are characterized by a reduction in lung size or an
increase in lung stiffness resulting in a decrease in the maximum volume of air
that can be moved in and out of the lung such as with interstitial lung disease,
neuromuscular disorders (e.g., amyotrophic lateral sclerosis or myopathic
disorders), sarcoidosis, pleural disorders, or abnormalities of the chest wall.
Disorders of the pulmonary vasculature include pulmonary embolism,
pulmonary hypertension, and pulmonary venoocclusive disease
– Restrictive lung disease is a major issue that should be aggressively managed
similar to other MNDs. During the progressive phase of the disease ventilatory
muscle strength may fall rapidly, whereas there is stability thereafter.
Importantly, although motor neuron loss does stabilize, strength and vital
capacity can often decrease during periods of growth.55 Pulmonary disease is
the main source of mortality and includes complications of muscle weakness
leading to impaired ventilation and secretion management or secondary
complications, such as pneumonia related to aspiration.
– Effective ventilation requires sufficient movement of air into and out of the
lungs by way of activation of breathing musculature including the diaphragm
and intercostal muscles, as well as a compliant rib cage to allow chest
expansion. In some myopathies, including Duchenne muscular dystrophy, most
congenital muscular dystrophies, severe forms of many congenital myopathies,
and myotonic muscular dystrophy, the diaphragm and intercostal muscles may
be weakened. Additionally, fibrosis of this musculature as seen in dystrophic
myopathies can result in a functional restrictive lung disease because the
– chest has limited expansion during inspiration. The patient and family should be educated in
the potential changes in respiratory function including discussion of management options and
their wishes for level of treatment. Symptomatic hypoventilation will often be reported as
snoring, insomnia, daytime somnolence, fatigue, drowsiness, depression, impaired cognitive
function, and morning headaches.50 As ventilation capacity declines, the forced vital capacity
(FVC) decreases resulting in increased serum CO2 levels. The increased partial pressure of CO2
results in a “right shift” of the oxygen dissociation curve such that there is a lower affinity of
hemoglobin to oxygen, which is why monitoring oxygen saturation via pulse oximetry alone is
an inadequate assessment of ventilatory status. Noone pulmonary test can predict the
development of hypoventilation or morbidity, thus regular monitoring of multiple parameters
is frequently used to detect respiratory compromise. A screening montage may include
oxygen saturation, FVC, peak cough flow, maximal inspiratory pressure (MIP), maximal
expiratory pressure (MEP), and end-tidal CO2 every 6 to 12 months or more frequently if
clinically indicated.
Treatment

In extrinsic restrictive disease the parenchyma of the lung

is normal and gas exchange is preserved, meaning that
treatment is usually respiratory muscle training and
mechanical ventilatory support as needed
Treatment

– Intrinsic restrictive lung diseases there may be a profound

associated hypoxemia from severely decreased diffusion capacity
of scarred lung tissue.
– Patients with parenchymal restrictive disease classically have
severe hypoxemia and may need high-flow supplemental oxygen.
Patients with end-stage intrinsic restrictive disease can have
ventilatory failure with hypercarbia and hypoxemia and may be
refractory to ventilatory support, and lung transplantation is then
often the only remaining treatment option
Tonelli et al. BMC Pulmonary Medicine (2017) 17:130
PR program consisted of:
– 6-h/week individually exercise training, including endurance training for upper and
lower limbs, 2 session of breathing techniques lasting 30 min for 4 to 5 times per
week and 3 sessions of group education per week. Exercise training consist of
aerobic (treadmill, stationary bikes) and resistance training (light weights, resistance
bands) and included supervised cycle and supported arm ergometry, and leisure
walking used as outcome measures, suitable for a pre-to-post comparison.
– Breathing training consisted of breathing techniques (controlled and diaphragmatic
breathing), pacing and energy conservation
– Supplemental oxygen was delivered to maintain normal level of oxygen saturation
– The educational topics included medication and
– Oxygen use, nutrition, panic control and relaxation techniques, as well as
psychosocial support and issues of palliation and/or end-of-life related to the
disease progression
– If needed, patients received psychosocial support
Seong-Woong Kang
Yonsei Medical Journal Vol. 47, No. 3, pp. 307 - 314, 2006

– Inspiratory muscle aids (ventilation assist)

Mechanical ventilators used in non-invasive ventilation
Glossopharyngeal breathing (GPB)/Frog breathing
– Expiratory muscle aids
Assistive coughing
Cough assist device