Chronic Obstructive Airway Disease

Interstitial Lung Disease

Chronic Kidney Disease

PCP 702 presentation

What is COPD?

Condition characterized by irreversible airflow obstruction resulting from thickening, narrowing and destruction of the small airways and structural damage to the lung parenchyma.

It encompasses 2 conditions: Chronic bronchitis (chronic productive cough for 3 months for 2 consecutive years) and Emphysema (abnormal permanent enlargement of the air spaces distal to the terminal bronchioles, accompanied by destruction of their walls)

COPD is treatable and preventable and also has some significant extra-pulmonary effects.

Epidemiology

According to the latest WHO estimates (2004), currently 64 million people have COPD and 3 million people died of COPD (5% of all deaths globally).

It is known that almost 90% of COPD deaths occur in low- and middle-income countries.

COPD used to be more common in men, but now affects men and women almost equally.

WHO predicts that COPD will become the third leading cause of death worldwide by 2030.

Etiologies Cigarette smoking

Environmental factors

Airway Hyper responsiveness

Alpha1-antitrypsin deficiency

Intravenous drug use

Immunodeficiency syndromes

Vasculitis syndrome

Connective tissue disorders

Salla disease

Pathophysiology

Clinical ManifestationsSymptoms Chronic productive cough- worse in the morning with small amount of colorless sputum Breathlessness Wheezing- especially during exertion & exacerbations Recurrent RTI

Signs Increased RR Use of accessory respiratory muscles Cyanosis & elevated JVP- in advanced disease Barrel chest Wheezing Symmetrically reduced breath sounds Hyper resonant chest on percussion Prolonged expiration

Systemic Effects- muscle weakness, edema, weight loss, osteoporosis, inc. circulating inflammatory mediators.

To distinguish between the 2 types of COPD:

Chronic Bronchitis Emphysema

Productive cough with gradual progression to intermittent dyspnoea, frequent and recurrentpulmonary infections and progressive cardiac/respiratory failure with oedema and weight gain

Long history of progressive dyspnoea with late onset of non productive cough, occasional mucopurulent relapses and eventual cachexia and respiratory failure

• Obese• Freq. cough &

expectoration• Use of accessory resp.

muscles• Coarse rhonchi and

wheezing on auscultation• Signs of heart failure-

oedema & cyanosis

• Thin & barrel chested• Little or no cough• Pursed lips and use of

accessory resp. muscles• Chest is hyper resonant• Wheezing on

auscultation• Distant heart sounds on

auscultation

Investigations

FBC, UECr- PCV

ABG- PaO2 +/- hypercapnia

PEFR- reduced

Lung Function Tests- reduced FEV1 and FEV1/FVC ratio

CXR- usually normal in early disease, but later demonstrates hyper expansion with a low lying flat hemi-diaphragm, long cardiac silhouette and increased retrosternal airspace

ManagementAim: to improve a patient’s functional status and quality of life by preserving optimal lung function, improving symptoms, and preventing the recurrence of exacerbations. Educate the patient about the disease and to encourage his or her

active participation in therapy. STOP smoking Nutritional support Bronchodilators-i)Short- acting administered prn or bd either :Beta-agonist eg. Salbutamol 1-2 puffs inhaled ORAnticholinergic eg. Ipatropium bromide 1-2 puffs inhaledii)Long acting bronchodilators(salmetrol or tiatropium bromide) are not on the EDL in Fiji

Management (cont.)

Corticosteroids

Inhaled eg. Beclomethasone or oral eg. Prednisolone

Methyxanthines eg. Theophylline

Immunizations for influenza & pneumococcal

Pulmonary Rehabilitation Programs

Home O2

Surgery

Management (cont.)

Acute Exacerbations1. Investigate and treat for precipitant2. Determine need for admission Mild: treat as an OP with inc freq of salbutamol and short course oral

prednisolone More severe:- admit and administer O2 maintaining a Sao2 ≥90% (watch for signs of hypercapnia)- administer salbutamol nebs 1-6 hrly ± ipratropium bromide nebs 4-6 hrly-Prednisolone oral 25-50mg daily for 7-14 days OR IV hydrocortisone 100mg 6hrly-Amoxicillin 500mg tds for 5 days if evidence of infection-Physiotherapy to aid sputum expectoration

Anatomythe lungs are ingeniously constructed to carry out their cardinal function: the exchange of gases between inspired air and blood.

Alveolar epithelium (type 1 & 2 pneumocytes) >> Alveolar basement membrane >> surrounding interstitial fluid >> Capillary Basement membrane >> Capillary endothelium

Restrictive lung diseases may be divided into the following groups:

Intrinsic lung diseases (diseases of the lung parenchyma)

Extrinsic disorders (extra-parenchymal diseases)

Intrinsic Lung DiseasesThese diseases cause either:

Inflammation and/or scarring of lung tissue (interstitial lung disease)

or

Fill the air spaces with exudate and debris (pneumonitis).

These diseases are classified further according to the etiological factor.

What are interstitial lung dxs? ILD’s are a heterogenous group of disorders characterized

predominantly by inflammation and fibrosis of pulmonary connective tissue, principally the most peripheral and delicate interstitium in the alveolar walls

Diffuse parenchymal lung disease (DPLD)

Presentation and natural history of these disorders may differ widely but they are frequently considered collectively as they share similar symptoms, physical signs, pulmonary function abnormalities and radiological changes

- these disorders account for about 15% of non-infectious diseases seen by pulmonary physicians.(USA)

PathophysiologyIntrinsic lung diseases:

Diffuse parenchymal disorders cause reduction in all lung volumes.

This is produced by excessive elastic recoil of the lungs.

Expiratory flows are reduced in proportion to lung volumes.

Arterial hypoxemia is caused by ventilation/perfusion mismatch.

Impaired diffusion of oxygen will cause exercise-induced desaturation.

Hyperventilation at rest secondary to reflex stimulation.

AetiologyInhaled substances• inorganic-Silica-asbestosis -Berylliosis•organic-Hypersensitivity pneumonitis

Drug inducedAntibioticsChemotherapeutic drugsAnti-arrhythmic drugs

Connective tissue dxSystemic sclerosisPolymyositisDermatomyositisSystemic lupus erythematosusRheumatoid arthritis

InfectionAtypical pneumoniaPneumocystispneumonia (PCD)TBChlamydia TrachomatisRespiratory synctialVirus

IdiopathicSarcoidosisIdiopathic pulmonary fibrosisHamman – RichsyndromeAntisynthetasesyndrome

MalignancyLymphatic carcinomatosis

Criteria Of DiagnosisMajor criteria Minor criteria

•Exclusion of known causes of ILD•Abnormal lung function (restriction & impaired gas exchange)•Hypoxia (rest or exercise)•Bi-basilar reticular abnormalities with minimal ground glass opacities on HRCT•Transbronchial lung biopsy or bronchoalveolar lavage showing no fts to support an alternative diagnosis

• 50 >Age •Insidious onset of otherwise unexplained dyspnea on exertion•Duration of illness >3 months•Bibasal inspiratory crackles.

Pathophysiology Allergen sensitivity/ infection inflammation

of distal air spaces release of chemical mediators (IL- 1, TGF- beta) & inflammatory cells

activate fibroblasts production and functionproliferation and excess elaboration of matrix

(collagen) , production of proteases and proteases inhibitors Fibrotic remodelling of distal airways

Investigations

Chest x-ray

Pulmonary function test (Spirometry, lung volumes and diffusing capacity of carbon monoxide [DLCO])

Pulse oximetry

High resolution CT

Complete blood count and differentials

LFT’s, RFT’s

Antinuclear antibody

Rheumatoid factor

Erythrocyte Sedimentation rate

Transbronchial/ surgical lung biopsy

Manifestation of ILD’s

Treatment Pharmocologicalo IV monthly cyclophosphamide with low dose oral glucocorticoids

(equivalent of prednisone < 10mg/day) o Liberal fluid intake is encouraged during therapyo Alternative regimen: Azatioprine + glucocorticoidso Montoring for renal crisis

o Supplemental oxygen therapy: via nasal cannula to prevent hypoxemia

o Drugs:o N-acetylcystein: slows the decline of lung function

is inhaled or delivered through a tube to the throato Anti-inflammatory drugs: prednisone & methylprednisolone (given as

adjuncts)o Antibiotics: Azithromycin & Levofloxacino Immunosuppressants: Azathioprine, Methotrexate, Cyclosporine,

Mycophenolate mofetil

Treatment Non pharmacological tx• Smoking cessation• Pulmonary rehabilitaion & Exercise therapy

- build aerobic fitness and maintain physical fitness• Avoidance of inhaled agents• Tube thoracostomy• Whole lung lavage• Lung transplantation: indicated to pts. With end- stage,

particularly fibrotic ILD and it shows to prolong life

Patient Education Vaccination and infection avoidance since pt. is on

immunosupressants Maintain hygiene

Specific measures There is ongoing controversy over the

role of specific treatments in subgroups of ILD due to a lack of good quality evidence for many of the specific treatments proposed.

Idiopathic pulmonary fibrosis-possible trial of azathioprine, prednisolone and N-actylcysteineLung transplantation is considered for suitable candidates

Connective tissue diseasesTx is based on disease severity (symptoms and LFTs) and longitudinal dx behaviour.Prednisolone (0.5 mg/kg/day), tapering to a maintenance dose of 10 mg/ day. This is often combined immunosuppressive agents(usually oral or IV cyclophosphamide)

Specific measures cont..Sarcoidosis- Tx is in only indicated if there is a progressive disease on radiology and lung function, or if there are significant symptoms or extrapulmonary disease.

Prednisolne (0.5 mg/kg/day) is usually first line treatment for 1 month, weaning to a maintenance dose that control symptoms and then continuing for up to 6 – 24 months

Hypersensitivity pnemonitis-Avoidance of the antigen is essential - prednisolone therapy may be required if there is severe or progressive disease.

Complications Superinfection in patients receiving

immunosuppressants.

Drug toxicity morbidity associated with ILD thus prevention and monitoring is crucial

Hemoptysis which is suggestive of vasculitis/ venooclussive dx

Respiratory failure/ Cor pulmonale / Right Heart failure

Death

Prognosis Mortality rates as high as 90% have been reported in children

who develop ILD when younger than 1 year (predominantly DIP); other studies have reported much better survival with conservative management.

Fan and Kozinetz reviewed the outcomes of 99 children with ILD over 15 years.[5]Survival rates at 24, 48, and 60 months after the appearance of initial symptoms were 83%, 72%, and 64%, respectively.

ILD and/or Idiopathic pulmonary fibrosis has a poor prognosis in older adults

Sarcoidosis which is more common in young adults, generally has a benign prognosis.

Primary Health Care Setting

Detailed history, medication and social history (smoking/occupation)

Physical examination

Investigations : CXR, Pulmonary function tests, Oximetry

Manage: O2, stop smoking, pulmonary rehabRefer to divisional hosp.

Conclusion Interstitial lung disease is a term encompassing a diverse range of lung

conditions that primarily affect the lung interstitium.

ILD should be considered in any person presenting with breathelessness or cough along with abnormal chest radiography or lung function testing.

High resolution computed tomography is the best imaging modality. Lung biopsy is often for diagnosis Referral to a respiratory specialist and involvement of multidisciplinary teams

are the mainstays of management.

Specific management varies according to the underlying diagnosis. General management strategies: pulmonary rehabilitation, smoking cessation,

oxygen therapy as required, and treatment of commonly associated diseases.

MANAGEMENT OF CHRONIC RENAL FAILURE

Definition

Presence of kidney damage (albuminuria ≥ 30mg/day)

Decreased kidney function (eGFR <60ml/min/1.73m2 ) for 3 or more months.

Chronic renal failure Chronic and irreversible loss of nephrons

Symptoms occur only after substantial loss of functioning kidney (>50%) and reflect the failure of its 3 major roles:

1. Regulation of fluid, electrolyte and acid-base balance (generally manifesting as fluid overload, hyperkalaemia and metabolic acidosis)

2. Excretion of nitrogenous waste products of protein catabolism (the accumulation of which leads to the many manifestations of ‘uraemia’)

3. Hormonal regulation, through production of erythropoietin (deficiency contributing to anaemia) and calcium homeostasis (disturbance leading to renal bone disease).

Patho-genesis of Progressive Renal Disease

Etiology

1. Metabolic disordersdiabetes mellitusamyloidosis

2. Renal vascular disordersrenal artery stenosisnephrosclerosis –hypertensionrenal vein thrombosis

3.Immunological disordersglomerulonephritispolyarthritis nodosasystemic lupus erytheromatosus

4.Infectionschronic pylonephritisTB

5.Primary tubular disordersnephrotoxins e.g.heavymetals,tetracyclins, analgesics

6. Urinary tract obstructionsrecurrent renal calculiBPH urethral stricture

7.Congenital disorderspolycystic diseasescongenital absences of kidney tissues.

Progression of Chronic Renal Failure

Initial pathogenetic insult Reduced renal reserve (loss of nephrons)

No change in urea or creatinine or homeostasis Renal insufficiency

mild elevation in urea and creatinine mild symptoms: eg. Nocturia & easy fatigability

Renal failure Disturbance in water, electrolyte and acid-base

metabolism Uraemic syndrome

Multiple dysfunctions of major organ systems End stage renal disease

Inability to sustain normal body function

Diagnosis1. History DM, HBP, nocturia, polyuria2. Physical exam 3. investigations

Blood tests Serum Creatinine and urea ( Cr cl using kockroft and gault equation) Electrolytes hyperkalemia, hyperphosphatemia, hypocalcemia FBC anemia lipid profile inc. cardiovascular risk ANA/ds DNA antibody SLE

Urine tests

Urinalysis – macroscopic , microscopic and dipstick – BROAD CAST : SPECIFIC FOR CRF (DILATED SURVIVING NEPHRONS). Non-specirfic : proteinuria, RBC

24hr urine tests – protein excretion, Cr cl Spot urine – ACR, UAC

Imaging

Ultrasound hydrinephrosis, retroperitoneum fibrosis, tumor, small echogenic kidney Plane x-ray ureteric obstruction, calculi Contrast urography ureteric obstruction, CT renal mass, cyst, calculi

Biopsy

For unclear dx

CKD Stages

Role of the GP

Responsible for managing CKD patients with stages 1,2,and3.

Principal goals of CKD management are:

-slow the rate at which nephrons are lost.

-reduce cardiovascular and renal risk

-early detection and management of CKD complicati0n

-avoidance of nephrotoxic medications and ensuring that dosage of other prescribed drugs are appropriate for the level of kidney function, avoiding contrast x-rays.

-and timely referral of CKD patients nephrologist.

Indications for referral

eGFR<30ml/min/1,73m2[stage 4 or 5 CKD]

Rapidly declining kidney function[>15% decr. in GFR over 3 months.

Significant proteinuria >1g/24hrs

Glomerular haematuria

Kidney impairment plus hypertension that proves difficult to control

Diabetes with kidney impairment or proteinuria/albuminuria

How to slow rate of progression of CKD

target blood pressure of less than 130/80 mm Hg.(KDOQI, JNC VII)

Target bp of less than 125/75 with proteinuria

RASI 1st choice of tx – renal protective Enalapril 5-15mg po bd

Edema – loop diuretics 1st choice tx

If serum creatinine levels increase more than 30% from baseline after adding RAS blockers, RAS blockers should be stopped.

Avoid in stage 3b, bilateral renal artery stenosis, or renal artery stenosis.

eGFR < 40 risk of hyperkalemia

1. Aggressive blood pressure control

2. Proteinuria goal One of the main factor for progression of ckd

Target proteinuria of <1000mg/day.

RASI 1st choice of tx.

non-dihydropyridine calcium channel blockers and aldosterone antagonist also have anti-proteinuric effect

3. Avoid further damage• Avoidance of nephrotoxins including intravenous (IV)

radiocontrast media, nonsteroidal anti-inflammatory agents (NSAIDs), and aminoglycosides.

• Ensuring that dosage of other prescribed drugs are appropriate for the level of kidney function.

4. Aggressive glycemic control. The American Diabetes Association (ADA) recommendations (target hemoglobin A1c [HbA1C] < 7%)

5. Treatment of hyperlipidemia. Total chol < 4.0 mmol/L

T2DM

IHD

Chronic renal failure

Dietary advice and Statin therapy simvastatin 5-40mg nocte

6. Protein restriction, salt, k, phosphate restriction.

7. Smoking cessation (nephrosclerosis)

8. Exercise and weight loss may reduce proteinuria

9. SNAPSS

10. All patient with CRF should be started on ACEI

Treating Complications of CKD

Treating edema and fluid overload

Usually develops in stage 5

Dec. GFR, RAS, aldosterone

Loop diuretic is the best diuretic to use

USE IV furosemide if severely edematous (poor absorption due to gut edema)

Adverse effect – diuresis related, hypersensitivity and ototoxicity.

Dietary sodium restriction <2g/day

drug Daily oral Moderate CKD Severe CKD

Furosemide 20-80 mg 80mg 200mg

Hyperkalemia Usually does not develop until the GFR falls to less than 20-25

ml/min/1,73m2.

Mild levels: low potassuim diet. Severe hyperkalemia can cause cardiac arrest! Avoid drugs that raise serum k eg NSAIDS, nonselective beta-

blockers Chronic or non-emergency control

Resonium (binds K+ in gut) 5-10g tds

Emergency treatment of high potassium (>7 mmol/l) with ECG changes –REFFER PATIENT First: - decrease myocardial excitability due to hyperkalemia

with IV Calcium chloride(500-1000mg infusion for 2-3 mins) or Calcium gluconate (1000mg infusion for 2-3 mins)(note: Calcium does not reduce potassium level; it is cardioprotectivein presence of high potassium)

Then, lower potassium level by facilitating the uptake of potassium into cells with 50ml of 50% dextrose and 10 units IV insulin -

Sodium bicarbonate (if acidosis)

Hypertension

Driven by volume expansion

3+ medications

1st line: enalapril 20mg BD

2nd line: HCT 50mg daily

3rd line: CCB, BB

Anemia in chronic renal failure

Type of anemia Normocytic normochromic

Pathogenesis of anemia Dec. erythropoetin, Uremia-induced platelet dysfunction,

nutritional deficiency.

Treatment Observation. FBC every 6/3 monthly for stage 3 and

below

Correct iron deficiency. Ferrous sulphate 300mg PO 6Qhr

Severely symptomatic refer - blood transfusion (Hb<7)

Recombinant erythropoetin ($$$$$$$)

Renal osteodystrophy Usually develops in stage 4 and 5 Patients with renal disease often develop low serum

calcium levels and bone disease Pathogenesis

The kidney is less able to produce calcitriol, and so is less able to reabsorb calcium

Gut reabsorption of calcium is also decreased Therefore serum calcium levels are low Low serum Ca++ levels lead to secretion of PTH PTH leads to resorption of calcium from the bones

If this goes on long enough, it isn’t particularly good for the skeleton!

Renal osteodystrophy

Treatment Hyperphosphatemia

Restrict dietary phosphate(milk, cheese, egg)

Phosphate binding drugs: calcium carbonate 500mg po/d

Hyperparathyroidism Calcitriol supplements: 0.125mg po/d

Hypocalcemia Calcium carbonate

Calcitiol

Caltrate 600mg/d

Metabolic Acidosis

Usually does not develop until stage 5

Kidney is the only organ to excrete hydrogen ions.

MA is associated with:

protein-energy malnutrition, loss of lean body mass, and muscle weakness.

Contributes to development of bone osteodystrophy bone acts as a buffer for excess acid, with resultant loss of mineral

tx- refer

- sodium-bicarbonate: 840 mg/d

ESRD - Uremia When do you get symptoms?

Stage 5 Associated with high blood urea nitrogen levels Less associated with serum creatinine

Distressing symptoms Anorexia, nausea and vomiting Lassitude, pruritis, twitching, platelet dysfunction

Major complications Uremic encephalopathy Uremic neuropathy Pericarditis Bleeding tendency Restless leg syndrome

Treatment Referral Renal replacement therapy

Vinaka