Lung infarction secondary to infected pulmonary arterial thrombosis in a patient with Eisenmenger's syndrome.

A case is presented where a patient with Eisenmenger's syndrome is successfully treated for infection of chronic pulmonary arterial thrombosis with good effect. The evidence for antibiotic and anticoagulant therapy in this patient group is discussed.

Restrictive lung defects: parenchymal, chest wall and neuromuscular.

Sarcoidosis is a multisystem condition which may affect a number of organs and, within the cardiopulmonary system, most commonly manifests as parenchymal, airway-centred, nodal, vascular or cardiac disease. Pleural involvement is rare, but well described, and often presents as pleural effusions or pleural thickening. Here, we present the first case of active sarcoidosis manifesting as bilateral pleural calcification. We highlight the importance of a nuanced understanding of pulmonary physiology when dissecting coexistent extrathoracic and intrathoracic pulmonary restriction. We demonstrate the value of positron emission tomography scanning for identification of sites of sarcoid activity, in this case the pleura, to ensure tissue confirmation of this rare but functionally important manifestation of disease. Sarcoidosis should be considered within the differential diagnosis for patients with pleural calcification, not explained by more common causes.

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.

Helicobacter pylori infection, host genetics and gastric cancer.

Helicobacter pylori infects half the world's population and is responsible for a considerable global health burden, including peptic ulcer disease and gastric cancer. The infection causes a chronic gastritis, the severity and distribution of which determine the clinical outcome. Bacterial, environmental and host genetic factors combine to define the degree of gastric damage. Most patients have a limited mild pan-gastritis with no significant clinical consequences. Antral-predominant gastritis is associated with high gastric acid output and an increased risk of duodenal ulcers. Corpus-predominant gastritis is associated with a reduction in gastric acid, multifocal gastric atrophy and an increased risk of gastric cancer. Host genetic factors are particularly important in defining the severity and extent of Helicobacter-induced gastritis. The most relevant and consistent genetic factors uncovered thus far are in the interleukin-1 and tumor necrosis factor-A gene clusters. These cytokines appear to play a key role in the pathophysiology of gastric cancer and their roles have been confirmed in animal models that mimic human gastric neoplasia. More genetic factors have also been uncovered and, with advancing technology, there is every prospect of defining a full genetic risk profile in the next decade. This will aid in targeting the testing and treatment of Helicobacter pylori, which offers a true opportunity to prevent and defeat this global killer.