Annick Bosseray

Hepatitis E and neuralgic amyotrophy: Five cases and review of literature.

Hepatitis E virus infection - mainly genotype 3 - is increasingly common in industrialized countries. Infection is usually asymptomatic, but cases of central or peripheral neurological symptoms with hepatitis E have been described. The most frequent is Guillain-Barre but somes cases of neuralgic amyotrophy have been described. In our center, since 2010, we have identified five cases of neuralgic amyotrophy associated with acute hepatitis E in immunocompetent patients. For all these patients, neuralgic amyotrophy was diagnosed with electromyogram and positive IgM for hepatitis E, and detectable HEV RNA in 4 of the cases. Including our patients, we count 26 cases in literature. The mean age of the patients was 44 years old, with a large predominance of males (88%). The disorder is bilateral and asymmetric in 69% of cases. Peripheral nerves other than the brachial plexus were affected in 6 patients (23%). In industrialized countries, any neuralgic amyotrophy, particularly if there is bilateral, asymmetric associated with involvement of nerves outside the brachial plexus, should lead physicians to consider a diagnosis of acute hepatitis E.

Unusual Evolution in Wegener's Granulomatosis: Recovery of Pulmonary Involvement While Renal Disease Progressed to End-Stage

A 54-year-old male patient was admitted for acute respiratory distress with fever. He was suffering from chronic sinusitis/rhinitis and had persistent otitis for the past 2 months before admission despite several antibiotics courses. He developed a complex pulmonary involvement (embolism and diffuse alveolar hemorrhage) with acute glomerular disease (proteinuria and hematuria but initially no renal failure). Clinical suspicion of Wegener’s granulomatosis was confirmed by the positive high titer of antineutrophil cytoplasmic antibodies (c-ANCA with antiproteinase 3 specificity) and despite a negative nasal biopsy. Treatment including cyclophosphamide and methylprednisolone intravenous pulses permitted pulmonary recovery over 4 weeks contrasting with the development of rapidly progressive glomerulonephritis and polyneuropathy of lower limbs. Renal biopsy showed pauci-immune crescentic and necrotizing glomerulonephritis. However, despite additional plasma exchanges, acute kidney injury worsened and the patient ended up in dialysis. Such a dissociated evolution was unexpected in this case since pulmonary and renal involvements reflected the same pathological process (small vessels vasculitis/capillaritis) and the same pathogenic mechanism (antiproteinase 3 autoantibodies).

Organising pneumonia and mesenteric plasmocytoma: a fortuitous association?

Pathophysiologically [1] organising pneumonia (OP) involves filling of the lumen of the distal air spaces by tissue containing inflammatory cells, fibroblasts and myofibroblasts in a relatively non-dense extracellular matrix. Its particular feature is its low content of type I collagen, probably explaining why the intra-alveolar structures are reversible. It has three clinical forms, the most classical of which involves multiple alveolar opacifications mimicking infectious pneumonia and often migratory (formerly known as bronchiolitis obliterans organizing pneumonia [BOOP]): the two other, rarer forms, are the pseudoneoplastic nodular forms (with single or multiple nodules) and the infiltrating form. The standard treatment is corticosteroid therapy to which they are generally sensitive. The occasional relapses can be treated with immunosuppressants including cyclophosphamide and azathioprine. The diagnosis requires a lung biopsy. OP can be cryptogenic (with no identified cause) or may occur in defined situations: medicinal products, infections and connective tissue diseases. Haematological diseases can occasionally be associated with OP. We describe the case of a 66-year-old female patient who presented with concomitant nodular OP and mesenteric plasmocytoma.