[The myeloproliferative neoplasms-related glomerulopathy]. / Les glomérulopathies associées aux néoplasies myéloprolifératives.

PURPOSE: Myeloproliferative neoplasms (MPN) are hematological disorders characterized by clonal expansion of one or more medullary lines. Renal complications are rare, chiefly as acute renal failure. Glomerular involvement is exceptional METHODS: We report on a retrospective multicenter case series of eight patients who presented with a glomerulopathy (GP) associated with MPN RESULTS: All GP were revealed by a major proteinuria frequently associated with nephrotic syndrome and oedema. Histology was mainly characterized by lesions of focal segmental glomerulosclerosis associated with increased mesangial cellularity. The pathophysiology is still unclear but platelet-derived growth factor (PDGF) and transforming growth factor-ß (TGF-ß), which play a central role in the MPN may be involved. A majority of patients developed chronic renal failure despite of a therapy intended to block the renin-angiotensin system CONCLUSION: Monitoring of proteinuria during the follow-up of MPN would allow earlier diagnosis of renal involvement. Further studies on a larger scale are needed to specify the pathophysiological mechanisms involved and the management of these complications.

[Preauricular lymphadenopathy related to Bartonella henselae]. / Adénopathies prétragiennes liées à Bartonella henselae.

INTRODUCTION: Cat scratch disease is characterized by adenitis with usually positive outcome. We reported two cases of cat scratch disease with preauricular involvement occurring in immunocompetent patients. OBSERVATIONS: Observation 1: a 28-year-old man had a recent onset of left cervical swelling, with a peripheral facial paralysis and liver cytolysis. Serologies for EBV, viral hepatitis, CMV, HIV and toxoplasma were negative. Node excision biopsy suggested granulomatous lymphadenitis and Bartonella henselae PCR on lymph node was positive. With doxycyclin for 3 months, associated with rifampicin for 15 days, abnormal liver function disappeared and facial paralysis improved. Observation 2: a 17-year-old man had parotid swelling associated with right posterior cervical lymphadenopathies associated with fever and profuse sweating. A large right preauricular lymphadenopathy with necrotic remodeling was visible on the CT-scan. Lymph fluid B. henselae PCR was positive. Positive outcome occurs after surgical drainage and short azithromycin treatment. CONCLUSION: Physicians should be aware of the rare preauricular localization of cat scratch disease and ask for contact with a cat. Parotid tumor localization, lymphoma or tuberculosis should be ruled out. Diagnosis is based on the B. henselae PCR. Outcome is often spontaneously positive but surgical treatment may be required.

[Recurrent pericarditis related to primary pericardial malignant mesothelioma]. / Péricardite récidivante : traquer le mésotheliome péricardique primitif.

INTRODUCTION: Most of recurrent pericarditis are idiopathic and only 15 to 20% have a specific diagnosis. Primary pericardial mesothelioma is a rare cause of recurrent pericarditis. Diagnosis can be challenging and antedates patient's death in only 10 to 20% of cases. Histology of mesothelioma and immunohistochemistry are mandatory for the diagnosis. Median of survival before using pemetrexed was about 6 months after diagnosis. CASE REPORT: We report the history of a 64-year-old woman for which repeated biopsy for recurrent pericarditis was necessary to diagnose a primary pericardial mesothelioma. The first biopsy had only found mesothelial hyperplasia. CONCLUSION: This case report highlights the necessity of repeat pericardial biopsy in the case of adverse outcome.

Is IgG4-Related Disease a Cause of Xerostomia? A Cohort Study of 60 Patients.

Objective. Immunoglobulin-G4-(IgG4-) related disease (IgG4 RD) is a fibrosing process characterized by a significant infiltration of IgG4-secreting plasma cells. IgG4 RD can affect almost all organs including salivary glands. Whether IgG4 RD plays a role in the development of sicca syndrome and particularly dry mouth syndrome remains to be investigated. Methods. We conducted a monocentric cohort study for two years to search for IgG4 RD features in patients with dry mouth syndrome using immunostainings of labial salivary gland specimens with anti-IgG4 antibody. Results. Among 60 patients presenting with dry mouth syndrome who underwent labial salivary gland biopsy, 18 showed positive immunostaining with the anti-IgG4 antibody including 4 patients with typical systemic IgG4 RD. Five also fulfilled criteria for Sjögren's syndrome. Conclusion. These findings suggest that clinical forms of IgG4 RD salivary involvement without salivary swelling may occur. This salivary involvement is probably overlooked in everyday practice and could represent a mild form of IgG4 RD.

[Nasosinusal and cervical sarcoidosis: a case series of three patients and literature review]. / Sarcoïdose nasosinusienne et cervicale traitée par infliximab : trois observations et revue de la littérature.

INTRODUCTION: Sinonasal sarcoidosis is difficult to treat. Infliximab seems to be useful in the treatment of sarcoidosis of the upper respiratory tract. CASE SERIES: We report three cases of sinonasal sarcoidosis in two women of 36 and 42-year-old and in a 64-year-old man. Resistance or dependence to corticosteroids and absence of efficacy of methotrexate therapy in one patient led to administer anti-TNFα therapy with infliximab. Outcome was favourable on sarcoid lesions but treatment was discontinued because of infectious complications and worsening of sarcoid chest involvement. CONCLUSION: This case series suggests that infliximab might be useful for the treatment of sarcoidosis with sinonasal involvement.

[Pituitary metastasis presenting as diabetes insipidus: a report of four cases and literature review]. / Diabète insipide révélateur de métastases hypophysaires : quatre observations et revue de la littérature.

INTRODUCTION: Pituitary metastases are a rare cause of central diabetes insipidus and usually complicate advanced cancers. CASE REPORTS: We report four cases in which diabetes insipidus revealed a metastatic stage of a lung (two cases) or a breast (two cases) cancer. One patient presented with a panhypopituitarism, three had diabetes insipidus including one with corticotroph insufficiency. In one case, the cerebrospinal fluid analysis showed a lymphocytic meningitis. On brain magnetic resonance imaging there was an absence of high intensity signal in the posterior pituitary lobe (one case) or an infiltration of the posterior lobe (three cases). CONCLUSION: Breast and lung malignancies are the most frequent causes of metastasis of the pituitary gland. In most cases there are often other metastatic locations; however insipidus diabetes can reveal the metastatic stage of the cancer. A pituitary biopsy may be necessary to obtain the diagnosis.