This was 76 year old lady of Pakistani origin who worked as an electrician exposing herself to silica.
The patient was admitted to hospital with shortness of breath, cough and fever over the last few days. The cough was non-productive, however as she was coughing a lot she developed chest pain. There was no history of a rash, coagulopathy or arthritis.
A chest radiograph (CXR) was performed which showed pulmonary congestion and right basal consolidation and was treated as an infective condition. The patient was discharged four days later with antibiotics and diuretics, with the plan to have a high resolution computed tomography (HRCT) scan, outpatient spirometry, followed by a chest clinic review as there seemed to be an underlying chronic lung pathology which needed to be investigated.
However, the patient was then readmitted three days later due to the worsening of her symptoms. A computerised tomography pulmonary angiography (CTPA) was performed which showed extensive peripheral opacities sparing the peri-hilar region and apico-basal gradient pattern. The findings were suggestive of eosinophilic lung disease or nonspecific interstitial pneumonia.
The patient carried out electronic soldering as her profession. During her work she was exposed to silica. She was also a passive smoker as her husband whom she lived with, was a smoker.
The bronchoalveolar lavage (BAL) from the right lower lobe laboratory analysis showed approximately 10mls of cloudy pink fluid. Microscopy result was benign respiratory epithelial cells, alveolar macrophages and scattered mixed inflammatory cells present. No malignant cells were seen. It is known in interstitial pneumonitis the diffuse alveolar damage is commonly caused by fibroblast invasion into the tissue and proliferation. TB culture and gram stain bacteriology from the BAL were also negative. However candida albicans was noted on mycology, which was thought to be likely a contaminant cause. Blood cultures and virology screen were also negative. However her (ANCA) perinuclear was mildly positive. Her serum ACE was 50u/l and IgE was elevated (512u/l). These results may occur in treated, inactive or relapsing microscopic polyangiitis (including its renal-limited variant), granulomatosis with polyangiitis and Churg-Strauss syndrome. Patients with systemic vasculitis in whom ANCA recur are more likely to relapse. It is also common in inflammatory bowel disease and other autoimmune diseases where its clinical significance is unclear. If ANA is present this may be a false positive result.
White cell count (WCC) 15 C-Reactive Protein (CRP) 77
7 days later WCC 8CRP 29
34 days later WCC 18 CRP 42
Bloods: on admission:
The echocardiogram revealed pulmonary hypertension. Autoimmune serology were sent (antinuclear antibodies, antineutrophil cytoplasmic antibodies, extractable nuclear antigen antibodies, IgE antibodies, aspergillus antibodies and precipitins, anti Ro, anti La, anti ribonucleoprotein, anti Smith, anti Jo1 and anti SCL-70 antibodies including anti nuclear antibody (liver) gastric parietal cell IgG antibody, smooth muscle IgG antibody, liver/kidney microsomal antibody and mitochondrial antibody) were all negative. A bronchoscopy was performed to exclude TB and fungal infections.
Imaging:
The extensive consolidation in the right mid zone and left base has increased since the previous film. There is loss of volume in both lungs. Atypical infection as well as acute, rapidly progressive pulmonary fibrosing alveolitis should be suspected.
The extensive parenchymal changes in both lungs are noted. No significant difference is identified compared to a previous film.
CXR Poor inspiratory effort with small volume lungs. Extensive interstitial and airspace shadowing in the right mid zone and both lung bases. There is a small left pleural effusion.
CXR Suboptimal aspiration with small volume lungs. There is consolidation in the right mid zone and left lower lobe. Small bilateral pleural effusions are present. A background of bilateral pulmonary congestion is present.
CT Pulmonary Angiogram:
CT imaging revealed:
Presence of extensive peripheral opacities of the lungs which are sparing the peri-hilar regions and show an apico-basal gradient pattern. The findings could be due to eosinophilic lung disease or non specific interstitial pneumonia (NSIP). Small bilateral pleural effusions seen. Reactive, borderline mediastinal lymph nodes seen. The heart size is normal and there is no pericardial effusion. The aorta and great vessels are normal in calibre. The central pulmonary arteries are patent with no evidence of embolus. The trachea and mainstem bronchi are patent. The oesophagus is normal in course and calibre. There is no pneumothorax. Scans through the upper abdomen are unremarkable. The osseous structures in the chest are intact. In comparison to CT PA of 37days prior there has been worsening of widespread patchy consolidation.
There are multifocal diffuse areas of consolidations bilaterally, involving all lobes. Air bronchograms are present. No pulmonary embolism.
No pleural/pericardial effusion.
No enlarged mediastinal or axillary lymph nodes. Small mediastinal lymph nodes noted.
These radiological findings were suggestive for eosinophilic lung disease or non-specific interstitial pneumonia (NSIP) as reviewed at the Lung Multidisciplinary Team meeting.
Differential diagnoses included infective causes, aspiration, drugs, autoimmune conditions particularly vasculitis such as Churg-Strauss syndrome and acute interstitial lung disease.
Radiological differential diagnosis included acute respiratory distress syndrome (ARDS), Churg Strauss and cryptogenic organizing pneumonia (COP).
On admission the patient was given a one week course of Co-amoxiclav which did not seem to improve the patient's cough. Therefore the patient was booked for follow-up in respiratory clinic with HRCT and outpatient spirometry.
One month later the patient returned to hospital with worsening of symptoms and was given two week course of intravenous (IV) Tazocin and Meropenem. The patient was discussed with the center of excellence in interstitial lung disease, who advised on stopping any possible triggers for interstitial pneumonitis (Aspirin and Omeprazole were the only possible medications reported in the literature to trigger Acute Interstitial Pneumonia (AIP) and adding Linezolid and Vancomycin for possible resistant organisms.
Following the interventions above the patient received a pulse therapy with methyl-Prednisolone of 1g IV for three consecutive days, followed by hydrocortisone IV and a prolonged course of Corticosteroids (Prednisolone 60mg daily, weaned to 30mg daily over a three month period). The patient's oxygen requirements reduced and she made good progress with the physiotherapists.
She was seen in the oxygen clinic three weeks after discharge requiring only 3L/min of oxygen 24 hours a day saturating at 98%. Another key element of the treatment consisted of high flow humidified heated oxygen therapy delivered via optiflow. This has helped prevent admission to ITU for intubation with a positive effect on acute hypoxemic respiratory failure and secretions management. Optiflow was delivered on the respiratory ward at a 60% Fio2, with a 60L/min flow and the patient's temperature was maintained at 37  celsius. Oxygen requirement improved over the 6 weeks admission to hospital, requiring 3L/min long term oxygen therapy (LTOT) on discharge. She had a further sleep study on 2L/min oxygen and was advised to continue Prednisolone 30mg daily for a further three months.