Owen J. Dempsey

Causes of pulmonary granulomas: a retrospective study of 500 cases from seven countries

Background The frequencies of various causes of pulmonary granulomas in pathological material are unknown, as is the influence of geographical location on aetiology. The aim of this study was to identify the causes of pulmonary granulomas in pathological specimens, to define their frequencies, and to determine whether these causes vary by geographical location. Methods 500 lung biopsies and resections containing granulomas were reviewed retrospectively by expert pulmonary pathologists from 10 institutions in seven countries. Fifty consecutive cases from each location were assigned a diagnosis based on histological features and available clinical/microbiological data. Results A specific cause was identified in 58% of cases (290/500), most commonly sarcoidosis (136, 27%) and mycobacterial or fungal infections (125, 25%). Mycobacteria were identified in 19% of cases outside the USA versus 8% within the USA. In contrast, fungi accounted for 19% cases in the USA versus 4% in other locations. Fungi were mostly detected by histology, whereas most mycobacteria were identified in cultures. In 42% of cases (210/500) an aetiology could not be determined. Conclusions Across several geographical settings, sarcoidosis and infections are the most common causes of pulmonary granulomas diagnosed in pathological specimens. Fungi are more commonly identified than mycobacteria in the USA, whereas the reverse is true in other countries. A definite aetiology cannot be demonstrated in more than a third of all cases of pulmonary granulomas, even after histological examination. These findings highlight the need to submit material for histology as well as cultures in all cases in which granulomatous disease enters the differential diagnosis.

Alveolar proteinosis with hypersensitivity pneumonitis: A new clinical phenotype

Background and objective:  The aim of the present study was to report the features of five patients with concurrent histopathological features of pulmonary alveolar proteinosis (PAP) and hypersensitivity pneumonitis (HP) and their high‐resolution CT (HRCT) appearances.

Cardiac sarcoid or arrhythmogenic right ventricular cardiomyopathy: A role for positron emission tomography (PET)?

We present a case of cardiac sarcoidosis of insidious onset mimicking arrhythmogenic right ventricular cardiomyopathy. Our patient initially presented with systemic sarcoidosis but later developed palpitations. The similarity in clinical presentation and cardiac magnetic resonance findings in both conditions posed a challenge in differentiating between the two in the absence of histological diagnosis. We highlighted the role of positron emission tomography in aiding a diagnosis.

Calcified pulmonary chondromas in Carney's triad

A 20-year-old woman presented with haematemesis. Endoscopy identified gastric ulceration. Chest imaging (figures 1 and 2) and subsequent surgical biopsy confirmed pulmonary chondromas (figures 3 and 4). These benign cartilaginous tumours, typically found in young women, can form part of ‘Carneys triad’ …

Misdiagnosis of common variable immune deficiency

We present details of a man who was originally diagnosed with sarcoidosis, based on a combination of nodal granulomatous inflammation and radiology confirming bilateral hilar lymphadenopathy with pulmonary infiltrates. The patient subsequently developed splenomegaly and idiopathic thrombocytopenic purpura (ITP) and, latterly, a severe cavitating pneumonia. Serum immunoglobulins were checked, confirming panhypogammaglobulinaemia, and his diagnosis was revised to common variable immune deficiency (CVID). CVID is a heterogeneous condition, which can mimic sarcoidosis with granulomatous organ involvement and is commonly complicated by autoimmune disorders, including ITP. Prompt recognition is important to allow early introduction of immunoglobulin replacement therapy to decrease infection frequency, reduce development of secondary disease complications and retard progression of tissue damage. Given the potential for misdiagnosis and delay in recognition of CVID, serum immunoglobulin measurement should be a first-line investigation in patients with suspected sarcoidosis, even if the presentation is ‘typical’. Current international sarcoidosis guidelines should be revised accordingly.

Is "nitrofurantoin lung"on the increase?

Gupta and Trautner suggest using nitrofurantoin as prophylaxis for recurrent urinary tract infections (UTIs) in non-pregnant women.1 They mention the risk of pulmonary toxicity (“nitrofurantoin lung”) associated with prolonged treatment but suggest that this occurs after years of treatment. In Grampian we have identified 13 patients in the past four years with nitrofurantoin lung, 12 of whom …

A puzzling pneumonia in a young immunosuppressed man

A 34-year-old Caucasian man with chronic renal failure on peritoneal dialysis presented with a 10-day history of productive cough, worsening dyspnoea and haemoptysis. He was a non-smoker, with no previous respiratory illnesses. There was no history of foreign travel or significant domestic or environmental exposures. He was feverish and tachypnoeic with scattered crackles throughout both lungs and mildly hypoxic (oxygen saturation 92% on air). He had no peripheral oedema. His primary renal disease was focal segmental glomerulosclerosis with frequently relapsing nephrotic syndrome since childhood. Medication included long-term immunosuppressive therapy with tacrolimus and corticosteroids (5 mg prednisolone), and he had received cyclophosphamide and levamisole during childhood. He had started fluoxetine hydrochloride 20 mg daily for depression 6 months earlier. Renal transplantation was being considered. A chest radiograph showed bilateral patchy consolidation. Pneumonia was diagnosed clinically, and he was treated with oral antibiotics and prednisolone dose doubled. Initially better, he was readmitted 2 weeks later with worsening breathlessness. He had raised …

Authors’ reply to Riddell and colleagues

We thank Riddell and colleagues for their important comments emphasising the role of lung transplantation in patients with idiopathic pulmonary fibrosis (IPF).1 Space precluded detailed discussion of this topic, but it is tackled in the latest IPF guidelines, which recommend considering transplantation in all patients without absolute contraindications, with …