Understanding Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis (IPF) is a chronic lung condition that causes progressive fibrosis of the interstitium of the lungs. Unlike other causes of lung fibrosis, IPF has no underlying cause. It is typically seen in patients aged 50-70 years and is more common in men.

The symptoms of IPF include progressive exertional dyspnoea, dry cough, clubbing, and bibasal fine end-inspiratory crepitations on auscultation. Diagnosis is made through spirometry, impaired gas exchange tests, and imaging such as chest x-rays and high-resolution CT scans.

Management of IPF includes pulmonary rehabilitation, but very few medications have been shown to be effective. Some evidence suggests that pirfenidone, an antifibrotic agent, may be useful in selected patients. Many patients will eventually require supplementary oxygen and a lung transplant.

The prognosis for IPF is poor, with an average life expectancy of around 3-4 years. CT scans can show advanced pulmonary fibrosis, including honeycombing. While there is no cure for IPF, early diagnosis and management can help improve quality of life and potentially prolong survival.

Patient has exacerbation of COPD with maximal treatment for an hour. Remains acidotic with high PCO2 and respiratory acidosis. BTS guidelines suggest starting NIV.

Guidelines for Non-Invasive Ventilation in Acute Respiratory Failure

The British Thoracic Society (BTS) and the Royal College of Physicians have published guidelines for the use of non-invasive ventilation (NIV) in acute respiratory failure. NIV can be used in patients with COPD and respiratory acidosis with a pH of 7.25-7.35. However, patients with a pH lower than 7.25 require greater monitoring and a lower threshold for intubation and ventilation. NIV is also recommended for type II respiratory failure due to chest wall deformity, neuromuscular disease, or obstructive sleep apnea, as well as for cardiogenic pulmonary edema unresponsive to continuous positive airway pressure (CPAP) and weaning from tracheal intubation.

For patients with COPD, the recommended initial settings for bi-level pressure support include an expiratory positive airway pressure (EPAP) of 4-5 cm H2O, an inspiratory positive airway pressure (IPAP) of 10-15 cm H2O, a back-up rate of 15 breaths/min, and a back-up inspiration-to-expiration ratio of 1:3. These guidelines aim to improve patient outcomes and reduce the need for invasive mechanical ventilation.

The routine use of non-invasive ventilation in asthmatics is not supported by current guidelines.

Management of Acute Asthma

Acute asthma is classified by the British Thoracic Society (BTS) into three categories: moderate, severe, and life-threatening. Patients with any of the life-threatening features should be treated as having a life-threatening attack. A fourth category, Near-fatal asthma, is also recognized. Further assessment may include arterial blood gases for patients with oxygen saturation levels below 92%. A chest x-ray is not routinely recommended unless the patient has life-threatening asthma, suspected pneumothorax, or failure to respond to treatment.

Admission criteria include a previous near-fatal asthma attack, pregnancy, an attack occurring despite already using oral corticosteroid, and presentation at night. All patients with life-threatening asthma should be admitted to the hospital, and patients with features of severe acute asthma should also be admitted if they fail to respond to initial treatment. Oxygen therapy should be started for hypoxaemic patients. Bronchodilation with short-acting beta₂-agonists (SABA) is recommended, and all patients should be given 40-50mg of prednisolone orally daily. Ipratropium bromide and IV magnesium sulphate may also be considered for severe or life-threatening asthma. Patients who fail to respond require senior critical care support and should be treated in an appropriate ITU/HDU setting. Criteria for discharge include stability on discharge medication, checked and recorded inhaler technique, and PEF levels above 75% of best or predicted.

When investigating a suspected pulmonary embolism (PE), a low Wells score of ≤ 4 and a negative D-dimer result suggest that an alternative diagnosis should be considered and anticoagulation should be stopped. In this case, the patient’s symptoms and history of trauma suggest a musculoskeletal injury may be the cause of their chest pain and shortness of breath. An urgent CTPA would only be necessary if the Wells score was 4 or higher or if the D-dimer test was positive. As neither of these occurred, repeating the D-dimer test is unnecessary. Continuing anticoagulation without a confirmed PE would increase the risk of bleeding. If a PE is confirmed, anticoagulation with warfarin or a direct oral anticoagulant would be appropriate.

Investigating Pulmonary Embolism: Key Features and Diagnostic Criteria

Pulmonary embolism (PE) can be challenging to diagnose as it can present with a wide range of cardiorespiratory symptoms and signs depending on its location and size. The PIOPED study in 2007 found that tachypnea, crackles, tachycardia, and fever were the most common clinical signs associated with PE. To aid in the diagnosis of PE, NICE updated their guidelines in 2020 to include the use of the pulmonary embolism rule-out criteria (PERC) and the 2-level PE Wells score. The PERC rule should be used when there is a low pre-test probability of PE, and a negative PERC result reduces the probability of PE to less than 2%. The 2-level PE Wells score should be performed if a PE is suspected, with a score of more than 4 points indicating a likely PE and a score of 4 points or less indicating an unlikely PE.

If a PE is likely, an immediate computed tomography pulmonary angiogram (CTPA) should be arranged, and interim therapeutic anticoagulation should be given if there is a delay in getting the CTPA. If a PE is unlikely, a D-dimer test should be arranged, and if positive, an immediate CTPA should be performed. The consensus view from the British Thoracic Society and NICE guidelines is that CTPA is the recommended initial lung-imaging modality for non-massive PE. However, V/Q scanning may be used initially if appropriate facilities exist, the chest x-ray is normal, and there is no significant symptomatic concurrent cardiopulmonary disease.

Other diagnostic tools include age-adjusted D-dimer levels, ECG, chest x-ray, V/Q scan, and CTPA. It is important to note that a chest x-ray is recommended for all patients to exclude other pathology, but it is typically normal in PE. While investigating PE, it is crucial to consider other differential diagnoses and to tailor the diagnostic approach to the individual patient’s clinical presentation and risk factors.

Aspergilloma is a type of fungal growth that typically affects individuals with weakened immune systems or those who have pre-existing lung conditions like tuberculosis or emphysema. Common symptoms include coughing, fever, and coughing up blood. Treatment typically involves the use of antifungal medications like itraconazole.

Haemoptysis, or coughing up blood, can be caused by a variety of conditions. Lung cancer is a common cause, especially in individuals with a history of smoking. Symptoms of malignancy, such as weight loss and anorexia, may also be present. Pulmonary oedema, which is the accumulation of fluid in the lungs, can cause dyspnoea (shortness of breath) and is often accompanied by bibasal crackles and an S3 heart sound. Tuberculosis, a bacterial infection that primarily affects the lungs, can cause fever, night sweats, anorexia, and weight loss. Pulmonary embolism, a blockage in the pulmonary artery, can cause pleuritic chest pain, tachycardia (rapid heart rate), and tachypnoea (rapid breathing). Lower respiratory tract infections, such as pneumonia, typically have an acute onset and are characterized by a purulent cough. Bronchiectasis, a chronic lung condition, is often associated with a long history of cough and daily production of purulent sputum. Mitral stenosis, a narrowing of the mitral valve in the heart, can cause dyspnoea, atrial fibrillation, and a malar flush on the cheeks. Aspergilloma, a fungal infection that often occurs in individuals with a past history of tuberculosis, can cause severe haemoptysis and is characterized by a rounded opacity on chest x-ray. Granulomatosis with polyangiitis, an autoimmune disease, can affect both the upper and lower respiratory tracts and is associated with symptoms such as epistaxis (nosebleeds), sinusitis, dyspnoea, and haemoptysis. Goodpasture’s syndrome, another autoimmune disease, can cause haemoptysis and systemic symptoms such as fever and nausea, as well as glomerulonephritis (inflammation of the kidney’s filtering units).

Dealing with Choking Emergencies

Choking is a serious medical emergency that can be life-threatening. It occurs when the airway is partially or completely blocked, often while eating. The first step in dealing with a choking victim is to ask them if they are choking. If they are able to speak and breathe, it may be a mild obstruction. However, if they are unable to speak or breathe, it is a severe obstruction and requires immediate action.

According to the Resus Council, mild airway obstruction can be treated by encouraging the patient to cough. However, if the obstruction is severe and the patient is conscious, up to five back-blows and abdominal thrusts can be given. If these methods are unsuccessful, the cycle should be repeated. If the patient is unconscious, an ambulance should be called and CPR should be started.

It is important to note that choking can happen to anyone, so it is important to be prepared and know how to respond in an emergency. By following these steps, you can help save a life.

The treatment for Pneumocystis jiroveci pneumonia involves the use of co-trimoxazole, a combination of trimethoprim and sulfamethoxazole.

Pneumocystis jiroveci Pneumonia in HIV Patients

Pneumocystis jiroveci pneumonia (formerly known as Pneumocystis carinii pneumonia) is a common opportunistic infection in individuals with AIDS. Pneumocystis jiroveci is an organism that is classified as a fungus by some and a protozoa by others. Patients with a CD4 count below 200/mm³ should receive prophylaxis for PCP. Symptoms of PCP include dyspnea, dry cough, fever, and few chest signs. Pneumothorax is a common complication of PCP, and extrapulmonary manifestations are rare.

Chest X-rays typically show bilateral interstitial pulmonary infiltrates, but other findings such as lobar consolidation may also be present. Sputum tests often fail to show PCP, and bronchoalveolar lavage (BAL) is often necessary to demonstrate the presence of the organism. Treatment for PCP includes co-trimoxazole and IV pentamidine in severe cases. Aerosolized pentamidine is an alternative treatment but is less effective and carries a risk of pneumothorax. Steroids may be used if the patient is hypoxic, as they can reduce the risk of respiratory failure and death.

In summary, PCP is a common opportunistic infection in individuals with AIDS, and prophylaxis should be given to those with a CD4 count below 200/mm³. Symptoms include dyspnea, dry cough, and fever, and chest X-rays typically show bilateral interstitial pulmonary infiltrates. Treatment includes co-trimoxazole, IV pentamidine, and steroids if the patient is hypoxic.

Choosing the Next Step in Asthma Treatment: Addition of Inhaled Corticosteroids

According to the Scottish Intercollegiate Guidelines Network (SIGN)/British Thoracic Society (BTS) guidance, patients with asthma who have had an attack in the last two years, use inhaled β2 agonists three times or more a week, are symptomatic three times or more a week, or wake up one night a week should move to the next step of treatment. The preferred next step is the addition of inhaled corticosteroids, which should be titrated to the smallest effective dose while maintaining symptom control.

While an oral leukotriene-receptor antagonist is suggested as an alternative next step if the patient cannot take inhaled corticosteroids, it is not as effective as inhaled corticosteroids. Oral corticosteroids are not recommended as they have many side effects and are not necessary in this scenario.

An inhaled long-acting β2 agonist would be appropriate for the third step of treatment, but this patient should move to the second step, which involves inhaled corticosteroids and continuing as required salbutamol. Simply increasing the salbutamol dose would be inappropriate and not in line with guidance. This patient requires both preventer and reliever therapy to effectively manage her asthma.

Palliative care prescribing for pain is guided by NICE and SIGN guidelines. NICE recommends starting treatment with regular oral modified-release or immediate-release morphine, with immediate-release morphine for breakthrough pain. Laxatives should be prescribed for all patients initiating strong opioids, and antiemetics should be offered if nausea persists. Drowsiness is usually transient, but if it persists, the dose should be adjusted. SIGN advises that the breakthrough dose of morphine is one-sixth the daily dose, and all patients receiving opioids should be prescribed a laxative. Opioids should be used with caution in patients with chronic kidney disease, and oxycodone is preferred to morphine in patients with mild-moderate renal impairment. Metastatic bone pain may respond to strong opioids, bisphosphonates, or radiotherapy, and all patients should be considered for referral to a clinical oncologist for further treatment. When increasing the dose of opioids, the next dose should be increased by 30-50%. Conversion factors between opioids are also provided. Opioid side-effects are usually transient, such as nausea and drowsiness, but constipation can persist. In addition to strong opioids, bisphosphonates, and radiotherapy, denosumab may be used to treat metastatic bone pain.

Overall, the guidelines recommend starting with regular oral morphine and adjusting the dose as needed. Laxatives should be prescribed to prevent constipation, and antiemetics may be needed for nausea. Opioids should be used with caution in patients with chronic kidney disease, and oxycodone is preferred in patients with mild-moderate renal impairment. Metastatic bone pain may respond to strong opioids, bisphosphonates, or radiotherapy, and referral to a clinical oncologist should be considered. Conversion factors between opioids are provided, and the next dose should be increased by 30-50% when adjusting the dose. Opioid side-effects are usually transient, but constipation can persist. Denosumab may also be used to treat metastatic bone pain.

According to NICE 2017 guidelines, if a patient with asthma is not effectively managed with a SABA + ICS, the first step should be to add a LTRA rather than a LABA.

The management of asthma in adults has been updated by NICE in 2017, following the 2016 BTS guidelines. One of the significant changes is in ‘step 3’, where patients on a SABA + ICS whose asthma is not well controlled should be offered a leukotriene receptor antagonist instead of a LABA. NICE does not follow the stepwise approach of the previous BTS guidelines, but to make the guidelines easier to follow, we have added our own steps. The steps range from newly-diagnosed asthma to SABA +/- LTRA + one of the following options, including increasing ICS to high-dose, a trial of an additional drug, or seeking advice from a healthcare professional with expertise in asthma. Maintenance and reliever therapy (MART) is a form of combined ICS and LABA treatment that is only available for ICS and LABA combinations in which the LABA has a fast-acting component. It should be noted that NICE does not recommend changing treatment in patients who have well-controlled asthma simply to adhere to the latest guidance. The definitions of what constitutes a low, moderate, or high-dose ICS have also changed, with <= 400 micrograms budesonide or equivalent being a low dose, 400 micrograms - 800 micrograms budesonide or equivalent being a moderate dose, and > 800 micrograms budesonide or equivalent being a high dose for adults.