Common variable immunodeficiency with granulomatous-lymphocytic interstitial lung disease and preceding neurological involvement: a case-report.

BACKGROUND: Common variable immunodeficiency (CVID) is a group of heterogeneous primary immunodeficiencies characterised by a dysregulated and impaired immune response. In addition to an increased susceptibility to infection, it is also associated with noninfectious autoimmune and lymphoproliferative complications. CVID is rarely associated with neurological complications. Pulmonary involvement is more common, and patients can develop an interstitial lung disease known as granulomatous-lymphocytic interstitial lung disease (GLILD). CASE PRESENTATION: A 50-year-old Caucasian female with a history of Evans syndrome (idiopathic thrombocytopaenic purpura and autoimmune haemolytic anaemia) and hypogammaglobulinaemia initially presented to the neurology clinic with marked cerebellar ataxia and headaches. Following extensive investigation (which included brain biopsy), she was diagnosed with neuro-sarcoidosis and her symptoms resolved following treatment with immunosuppressive therapy. Over the following 10 years, she was extensively investigated for recurrent pulmonary infections and abnormal radiological findings, which included pulmonary nodules, infiltrates and splenomegaly. Subsequently, she was referred to an immunology clinic, where immunoglobulin replacement treatment was started for what was ultimately considered to be CVID. Shortly afterwards, evaluation of her clinical, radiological and histological findings at a specialist interstitial lung disease clinic led to a diagnosis of GLILD. CONCLUSION: CVID is a condition which should be suspected in patients with immunodeficiency and recurrent infections. Concomitant autoimmune disorders such as haemolytic anaemia and immune thrombocytopenia may further support the diagnosis. As illustrated in this case, there is a rare association between CVID and inflammatory involvement of the neurological system. Respiratory physicians should also suspect CVID with associated GLILD in patients with apparent pulmonary granulomatous disease and recurrent infections. In addition, this case also highlights the challenge of diagnosing CVID and its associated features, and how the definitive exclusion of other pathologies such as malignancy, mycobacterial infection and lymphoma is required as part of this diagnostic process.

Helical CT with variable target noise levels for dose reduction in chest, abdomen and pelvis CT.

OBJECTIVES: To evaluate a contiguous helical CT protocol with two different target noise levels in chest/abdomen/pelvis CT. METHODS: 41 patients (study group) underwent a helical scan (P1) with two different target noise levels (SDs), SD = 16 for chest and SD = 13 for abdomen/pelvis. Two further protocols were planned but not executed: a single helical scan with only one SD (SD = 13) for the entire scan range (P2), and two separate helical scans overlapping over the liver and same SD settings as for P1 (P3). All DLPs were recorded. Image quality was assessed qualitatively and quantitatively on all scans. The control group consisted of 40 patients, was scanned with protocol P3 and analysed using the same metrics. RESULTS: DLPs (mean/SD) for P1, P2 and P3 were 859.5/392.9, 1040.2/510.5 and 1027.4/469.4, respectively. P1 offered a mean dose reduction of 17.4% compared to P2, and 16.3% compared to P3 (both p < 0.001). There were no differences in image quality between both patient groups (p > 0.3). CONCLUSION: Contiguous helical scanning of the chest/abdomen/pelvis with variable target noise levels results in approximately 17% dose reduction if compared to a single acquisition with only abdominal dose settings or two separate acquisitions of the chest and abdomen/pelvis. KEY POINTS: • Low dose chest and standard abdomen CTs can be combined. • Variable SD CT scanning allows for radiation dose reduction. • Variable SD CT scanning maintains image quality.