Adult-onset Still's disease after SARS-Cov-2 infection.

Adult-onset Still's disease (AOSD) is an uncommon inflammatory disorder. AOSD and SARS-Cov-2 infection share clinical and laboratory features, including systemic inflammation. A 19-year-old woman had prolonged fever for 3 weeks, joint pain, and biological inflammatory syndrome. Post COVID-19 AOSD was diagnosed. SARS-Cov-2 infection induces many inflammatory diseases including AOSD.

Primary Sjögren's syndrome complicated by anti-neutrophil cytoplasmic antibody-mediated crescentic glomerulonephritis.

Ocular and oral dryness are the hallmark of Sjögren's syndrome (SS). However, SS can be associated with a variety of complications, affecting organs such as the liver, kidneys, lungs, muscle, and nervous system. Renal involvement has been usually in the form of tubulointerstitial nephritis. However, glomerulonephritis is rare in primary SS. We report three clinical cases of SS with anti-neutrophil cytoplasmic antibody-mediated crescentic glomerulo-nephritis treated with prednisone and cyclophosphamide, with favorable outcome.

[Panniculitis in patient undergoing treatment for dermatomyositis with methotrexate]. / Panniculite au cours d'un traitement d'une dermatomyosite par du méthotrexate.

Panniculitis is a rare cutaneous manifestation of dermatomyositis (DM). The appearance of panniculitis during treatment with methotrexate (MTX) is exceptional and has only been described in 3 cases. We report a case of a 50-year-old woman suffering from DM since 1997 who was treated with corticosteroids showing favorable clinical and biological evolution. When a relapse occurred 2 years later, she was treated with higher-dose of corticosteroids in combination with a 7,5 mg weekly dose of methotrexate. The evolution was rapidly favorable. Eighteen months later, the patient had multiple subcutaneous nodules on limbs and buttocks. Anatomopathological examination showed panniculitis. There was no evidence supporting progression in DM. Prednisone dose was increased to 0.5 mg/kg/day, always in combination with MTX, without any clear signs of improvement. MTX treatment was stopped and the cutaneous lesions completely disappeared in 2 months without any relapse. This objective response lasted for 42 months. Our observation is particular given the occurrence of panniculitis in a patient undergoing treatment for dermatomyositis with methotrexate and illustrates the difficulties in the diagnosis. This entity must be known despite its exceptional nature since cutting off MTX treatment generally induces the disappearance of subcutaneous nodules.

Pleural myelomatous involvement in multiple myeloma: five cases.

Pleural myelomatous involvement in multiple myeloma (MM) is rare, occurring in less than 1% of cases. We retrospectively studied five cases of patients with MM who developed myelomatous pleural effusions. Three men and 2 women with a mean age of 61 years presented with myelomatous pleural effusion. The pleural fluid electrophoresis revealed a peak of IgG in three cases, of IgA in one case, and of lambda light chains in one case, which were identical to that in the sera of the patients. Detection of typical plasma cells in pleural fluid cytology was contributive, and histologic confirmation by pleural biopsy was positive in four cases. Treatment consisted of chemotherapy. The clinical outcome was initially good, but relapses occurred in all cases early and were complicated by fatal infections. Myelomatous pleural effusion is a rare affection. It is usually a late complication associated with poor prognosis.

Pseudotumoral brain lesion as the presenting feature of primary Sjögren's syndrome.

BACKGROUND: The frequency and type of central nervous system involvement in primary Sjögren's syndrome (pSS) remain controversial. Brain magnetic resonance imaging (MRI) abnormalities in pSS are usually discrete hyperintense areas in the white matter. Tumefactive brain lesions have been rarely reported. CASE REPORT: We describe a 31-year-old woman who exhibited transcortical motor aphasia, hemiparesis and partial motor seizures as the initial manifestation of pSS. Brain MRI revealed a large frontoparietal lesion extending into the corpus callosum. The patient had spontaneous recovery and developed sicca symptoms 6 months after onset. Primary SS was diagnosed on the basis of clinical features, abnormal Schirmer test findings, high levels of anti-La/SSB antibodies and positive salivary gland biopsy results. CONCLUSION: The present case suggests that a pseudotumoral brain lesion can occur as an initial symptom of pSS.

RORC and Foxp3 axis in cerebrospinal fluid of patients with neuro-Behçet's disease.

Neurological manifestations are present in 5% to 30% of patients with Behçet's disease (BD). Neuro-Behçet's Disease (NBD) is hypothetically caused by T helper (Th) cells, which development is dependent on the expression of lineage-specific transcription factors. Cerebrospinal fluid (CSF) mRNA expression of TBX21, GATA3, RORC, FOXP3 and EBI3 were assessed in 18 NBD patients and 26 controls disease [16 noninflammatory neurological disease (NIND) and 10 headache attributed to Behçet's disease (HaBD)]. Expression of TBX21 (Th1), RORC (Th17) and Foxp3 (Treg) were increased in NBD patients compared to HaBD and NIND patients. EBI3 and Th2-associated GATA3 expressions were found to be decreased (P<0.0001 and P<0.0001) in NBD patients. Analysis of transcription factor ratios, revealed an increase in the RORC/FOXP3 and TBX21/GATA3 ratios in NBD patients (P<0.0001; P<0.0003). Our findings indicate that both Th1 and Th17 mRNA expressions involving a possible impairment of Treg cells. This might play a role in CSF-NBD inflammation, permitting activation of harmful T cell subpopulations. The TBX21/GATA3 and RORC/FOXP3 ratios dysregulations in NBD are consistent with those reported in other inflammatory diseases and indicating the plasticity existing between Th1, Th17 and Treg cells during inflammation.

Peritoneal amyloidosis: unusual localization of gastrointestinal amyloidosis.

Amyloidosis is a rare disease that results from the extracellular deposition of amorphous fibrillar protein. It is usually observed in a systemic form. Gastrointestinal involvement is frequent but peritoneal localization is unusual. A 43-year-old male was investigated for nephritic colic. Morphologic explorations revealed small intestine agglomerans in the periumbilical region, infiltration of peritoneal fat and multiple coelio-mesenteric lymph nodes. There were no clinical or biological abnormalities and endoscopic examinations were normal. The patient then underwent an exploratory laparoscopy. Macroscopically false membranes were seen throughout the peritoneum and small bowel without ascites. Anatomopathologic examination diagnosed peritoneal amyloidosis. After several investigations a diagnosis of a primary peritoneal amyloidosis was confirmed. The patient was treated with melphalan and prednisone with a favorable outcome. Our case illustrates a particular presentation of peritoneal amyloidosis. Despite improved imaging methods, peritoneal biopsy remains essential for diagnosis.

[Pericarditis in systemic lupus erythematosus: prevalence and clinical and immunologic characteristics]. / Péricardites lupiques : prévalence, caractéristiques cliniques et immunologiques.

PURPOSE: Our study sought to determine the frequency and clinical and immunologic characteristics of pericarditis in patients with systemic lupus erythematosus (SLE). METHODS: We retrospectively analyzed the files of all patients with SLE diagnosed between 1987 and 2005. The study included only those patients who had undergone echocardiography. They were divided in two groups according to whether or not they had pericarditis, and their clinical and immunologic characteristics and outcome were compared. RESULTS: Our records found 180 patients with SLE, 97 of whom had had echocardiography. The frequency of pericarditis was 39%. The average age of the 34 women and 4 men with pericarditis at its onset was 36.4 years. There were 59 SLE patients without pericarditis, 56 women and 3 men with an average age of 28.8 years. Pericarditis was frequently associated with pleural effusions. The prevalence of anticardiolipin antibodies was significantly lower in the group of patients with pericarditis. CONCLUSION: Pericarditis is the most common cardiac manifestation in SLE. Its frequency in this sample of Tunisian patients was similar to that described in different ethnic groups. The association between pericarditis and pleuritis is standard. Anticardiolipin antibodies, commonly positive in valve diseases, were less frequent in our patients with pericarditis.

Hyperhomocysteinemia is associated with deep venous thrombosis of the lower extremities in Tunisian patients.

OBJECTIVES: To test the association between hyperhomocysteinemia (HHC) and deep venous thrombosis (DVT) of lower extremities in Tunisians. DESIGN AND METHODS: This case-control study included 90 patients with DVT of the lower extremities and 160 healthy controls. Plasma homocysteine, vitamin B(12) and folate were determined using immunoenzymatic methods. Logistic regression models were performed to test whether the association between HHC and DVT is independent and to precise determinants of HHC in DVT patients. RESULTS: Plasma total homocysteine concentrations were significantly higher in patients with DVT (17.4+/-11.5 micromol/L) and in patients with idiopathic DVT (15.2+/-6.4 micromol/L) as compared to controls (11.5+/-3.3 micromol/L). HHC was significantly associated (p<0.001) with all DVT (OR, 8.82; 95% CI, 3.96-19.6) as well as idiopathic DVT (OR, 7.40; 95% CI, 3.01-10.8). These associations persisted after adjustment for several thrombosis risk factors. In patients with DVT, HHC was related to folate and vitamin B(12) concentrations, but neither to the type of occurrence nor to the recurrence of DVT. CONCLUSION: HHC is independently associated with first DVT of lower extremities in Tunisians. Homocysteine should be assessed in patients with DVT and the effect of vitamin B supplementation should be tested among them.

[Melkersson-Rosenthal syndrome. Report of five cases and review of the literature]. / Syndrome de Melkersson-Rosenthal. A propos de cinq observations et revue de la littérature.

The goal of this work is to report five cases of Melkersson-Rosenthal syndrom with a literature review. It is a rare entity and is characterized in its complete presentation, by the association of reccurent orofacial swelling, peripheral facial palsy and plicated tongue. Incomplete forms are more frequent and more difficult to establish its diagnosis. This latter is based on major and minor clinical and histological critieria sorted in three levels. There is four forms of MRS. The pathogenesis of this syndrome is still unknown; treatment remains random. It is based on topical or systemic steroids with or without cheiloplastic procedure. We must think of MRS in presence of any recurrent peripheral facial palsy and/or chronic facial swelling.