Pulmonary manifestations in antisynthetase syndrome.

INTRODUCTION: Pulmonary manifestations are frequent in patients with antisynthetase syndrome which is a particular form of inflammatory myopathies. AIM: The aim of this study is to describe clinical features and long term outcome of interstitial lung disease in these patients. METHODS: This is a retrospective descriptive study in an internal medicine department. Patients with antisynthetase syndrome hospitalized from 2000 to 2014 were collected. RESULTS: There were nine patients; five women and four men. The mean age at diagnosis was 54.4 ±11.2 years. Interstitial lung disease was observed in all cases and revealed the disease in five cases. The more frequent aspect in high resolution computer thoracic scan was ground-glass opacities (n=8). Traction bronchiectasis and septal thickening were noted each one, in five cases. Honeycombing was observed in one case. Restrictive syndrome was noted in 4/4 cases. All patients received corticosteroids. Two patients were treated with methotrexate for myositis. Intravenous cyclophosphamide was used in five patients (at diagnosis for severe interstitial lung disease in three cases and after pulmonary function worsening in two other cases). Improvement was noted in seven patients. Two patients died after pulmonary symptom worsening and respiratory insufficiency. CONCLUSION: Interstitial lung disease in patients with antisynthetase syndrome may have a poor prognosis and should be treated at time. Altough the optimal therapy was not clearly established, corticosteroids are considered to be the first line therapy. Immunosuppressive agents as cyclophosphamide, azathioprine or methotrexate may be used in some cases.

Erdheim-Chester disease: A multisystem disease case illustration with rare manifestations and treatment challenges.

Erdheim-Chester disease is a rare multisystemic disease. A 50-year-old woman, presented with a recurrent pain and swelling of the left knee. Bone scintigraphy showed increased tracer uptake of peripheral skeleton. The computed tomography showed tissular infiltration in the retroperitoneum, around the vessels. Immunohistochemistry showed CD68 (+) and CD1a (-).

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.

Does lowering hyperhomocysteinemia by folic acid beneficial for oculo-Behcet's disease? A pilot study.

OBJECTIVE: To determine the effect of folic acid supplementation in Behçet's disease (BD) patients with ocular involvement associated with hyperhomocysteinemia (Hhcys). PATIENTS AND METHODS: 19 BD patients, all with uveitis and/or retinal vasculitis associated with Hhcys (plasma hcy > 15 micromol/l) were prospectively included. Patients were divided into 2 groups. Group 1 consisted of 11 patients that received folic acide (15 mg/d) in addition to their previous standard treatment. Group 2 included 8 patients treated only with their previous standard treatment. Visual acuity and uveitis attacks were assessed initially and monthly at each visit in all groups. Mean Visual acuity and frequency of uveitis attacks were evaluated quarterly. RESULTS: In group 1, the mean plasma hcys level was significantly lower after than before the treatment period (27.7 vs 13.1 micromol/l; p= 0.04) while it did not vary significantly in the same period in Group 2 patients. Frequency of uveitis attacks was significantly lower after than before treatment at each quarter in groupl and mean visual acuity in this group increased from 4.33 to 5.44 decimals. During the treatment period, the mean number of uveitis attacks, converted to frequency per 12 months were significantly lower in group 1 than in group 2. During this period, the VA slightly increased in group 1 and decreased in group 2 but the difference was not significant. CONCLUSION: Our results indicate that folates supplementation is useful for BD patients with Hhcys.

[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.

[Shulman syndrome. A report of 4 cases and review of the literature]. / Syndrome de Shulman. A propos de 4 cas avec revue de la litterature.

The goal of this study is to report four cases of Shulman syndrome with a literature review. Shulman syndrome is a rare disorder recently considered a systemic disease. Our first case shows woody induration of the buttock and trunk with features of morphea. The diagnosis of eosinophilic fasciitis, suspected on hypereosinophilia, was confirmed by histological findings of muscle biopsy. In the second and the third case, the induration affected arms and legs. Obiouvs streneous exercise was noted in the third patient. Those patients fullfiled the criteria of eosinopfilic fasciitis. Visceral involvement consisted on restrictive lung function defects on the second case and oesophageal hypokinesia in the third case. In the fourth case, there was a scleroderma-like on the extremitis with extension to abdomen. Erythrocyte sedimentation rate was normal. Histological findings confirm the diagnosis of eosinophilic fasciitis. All patients were treated with general steroids at high doses associated to cimetidine in the second patient. Once therapy ended, relapses occur in escond and third cases.

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.

Esophageal involvement in Behcet's disease.

Esophageal involvement in Behcet's disease is very rare, and normally is observed as aphtosis and esophagitis, but serious complications such as erosions, perforations, and stenosis may occur. We carried out this prospective study to evaluate the prevalence of esophageal involvement in Behcet's disease and to establish if routine endoscopy and/or manometry are necessary. Twenty-three patients who fulfilled the diagnostic criteria of the international study group for Behcet's disease were enrolled. None were taking drugs or had disease that might produce esophageal abnormalities or alter any existing changes due to the Behcet's disease itself. Twenty- three patients underwent esophagogastroduodenoscopy by a single observer. Esophageal biopsies were performed in thirteen patients and esophageal manometry in twenty. At the beginning of the study, the disease activity was defined by the presence of more than one symptom related to Behcet's disease, and upon the classification of Behcet's disease. Of the twenty three patients enrolled two were excluded from final analysis because of the presence of hiatus hernia. Thus, 13 men and 8 women, ranging in age from 20 to 63 years with a mean age of 36.2 years were included. Ten patients (47.6%) had active disease and four (19%) complained of upper gastrointestinal symptoms at the time of the study. Fourteen patients had endoscopic, manometric and/or microscopic abnormalities. Esophageal manometry was performed in twenty patients and was abnormal in 7 cases (35%). Esophageal biopsies were done in 13 patients and revealed histopathological abnormalities in 5 cases. Microscopic findings showed vasculities in one case, and non-specific inflammatory infiltration mainly consisting of neutrophils in 4 cases. Our results suggest that the prevalence of esophageal involvement in Behcet's disease is rather high and occur even in asymptomatic patients, but that this usually does not result in specific abnormalities.

[Benzylthiouracil induced ANCA-positive vasculitis]. / Vascularite à anticorps anticytoplasme de polynucléaires neutrophiles induite par le benzylthio-uracile.

INTRODUCTION: Several cases of vasculitis associated with antineutrophil cytoplasmic antibodies (ANCA) have been reported in patients treated with synthetic anti-thyroid drugs but only 2 cases have incriminated benzylthiouracil. OBSERVATION: A 36 year-old woman, 3 years after treatment with benzylthiouracil, rapidly developed progressive kidney failure, related to a pauci-immune extra-capillary glomerular nephropathy and necrotic vasculitis lesions. The search for p-ANCA was positive with anti-myeloperoxidase specificity. She was treated with corticosteroids and 6 monthly intravenous pulses of cyclophosphamide substituted by azathioprine. Renal failure and proteinuria significantly improved. However the high level of p-ANCA. CONCLUSION: ANCA vascularities are a rare but serious complication of treatment with synthetic thiouracile-type anti-thyroid drugs. The ANCA must be measured when confronted with a systemic manifestation during treatment.

[Hyperprolactinemia in systemic lupus erythematosus. A prospective study of 38 cases]. / L'hyperprolactinemie au cours du lupus erythemateux systemique. Etude prospective à propos de 38 cas.

OBJECTIVE: To study prospectively the serum prolactin concentrations among patients with systemic lupus erythematosus and their possible relationship to disease activity and manifestations. METHODS: Serum prolactin levels were measured by radioimmunoassay in 38 patients with systemic lupus erythematosus and 22 age matched controls. Patients with known secondary causes for hyperprolactinaemia, such as pregnancy, lactation, prolactinoma and taking medications known to induce prolactin secretion, were excluded from the study. Demographic, clinical and laboratory features of the patients were obtained. Patients were divided into two subgroups according to their disease activity. Mean prolactin levels from both groups were compared using student test, and prolactin from patients with systemic lupus erythematosus was correlated with variables of disease activity, including the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI). RESULTS: Mean prolactin levels were higher in patients with systemic lupus erythematosus (15.4) than healthy controls (9.83); however, the difference did not reach statistical significance (p=). Hyperprolactinemia was found in 24 patients with systemic lupus erythematosus and 5 controls. The frequency of hyperprolactinaemia in systemic lupus erythematosus group was higher than healthy controls. No significant difference in mean prolactin levels was found between patients with active versus inactive disease (18.9 vs 18.5). CONCLUSION: Hyperprolactinaemia occurred significantly in patients with systemic lupus erythematosus, but did not correlate with disease activity.

Comparison of clinical features of Behcet disease according to age in a Tunisian cohort.

Behcet's disease (BD) is a multisystemic inflammatory disease that occurs most often between the second and fourth decade of life. Patients have been reported during the first months of life and after 70 years. Our objective was to determine the clinical, paraclinical and genetic characteristics of BD in patients aged < 20 and > 40 years. We conducted a comparative retrospective study including patients with BD (Criteria of International Study Group on BD). Patients were divided into two groups: those < 20 years (Group one) and those > 40 years (Group two). The clinical, paraclinical and genetic (HLA) characteristics were determined and compared in the two groups. The data were compiled and analyzed using SPSS 11.0. Thirty totals of 430 patients were included. Group one included 81 patients (55 men and 26 women). Group two included 68 patients (45 men and 23 women). Cutaneous involvement (88.9 versus 76.5%; P=0.043), pseudofolliculitis (84 versus 64.5%; P=0.004) and vena cava thrombosis (11.11 vs 0%; P=0.004) were significantly more frequent in group one while joint involvements were more common in group two (57.4 versus 40.7%; P= 0.043). The frequency of erythema nodosum as well as ocular, vascular and neurological disorders was comparable between the two groups. Few studies in the literature have compared the clinical, paraclinical and genetic characteristics of BD, who had first symptom onset after 40 years of age. Late-onset BD, usually, affects both genders equally. According to present results, the frequency of severe organ involvement is equal regardless of age, except for vena cava thrombosis.

Characteristics of neurological manifestations of Behçet's disease: a retrospective monocentric study in Tunisia.

OBJECTIVE: The aim of the present study was to analyze demographic, clinical and genetic features of Behçet's disease patients with neurological involvement through a monocentric study of a homogenous group of hospitalized patients observed in the same department and to compare them with those of other ethnic and geographic groups. METHODS: Four hundred and thirty Behçet's disease (BD) patients were retrospectively studied. Diagnosis of BD was made according to the international study group for Behçet's disease criteria. Patients with neurological findings suggestive of involvement of the nervous system by BD were further studied according to clinical examination, laboratory tests and neuroradiological investigations. RESULTS: Neurological involvement was observed in 121 patients (28.1%). The mean age at neuro-Behçet's disease (NBD) onset was 29.7 years. Average disease duration of BD before neurological manifestations onset was 6.4 years. Male to female ratio was 1.8. Of the 121 NBD patients, parenchymal involvement occurred in 74 patients (61%). Among them 26 (21.4%) presented with brainstem involvement, 24 (19.8%) with hemispheric involvement and 2 (1.6%) with spinal cord involvement. Non-parenchymal NBD occurred in 47 patients (39%). Involvement of the main vascular structures (Vasculo-NBD) was the most common non-parenchymal NBD lesion found in 35 patients (28.9%) consisting of cerebral vein thrombosis (CVT) in 24 cases and cerebral arterial thrombosis in 11 cases. Forty-nine (40.5%) patients with NBD have been followed-up for a median of 3 years (range 1-19 years). Forty-one of them recovered well without significant residual disability, 5 patients made no improvement and are left with severe neurological impairments and 3 died. Male gender and CNS parenchymal lesions occurrence were significantly associated with a poorer prognosis. CONCLUSION: Clinical and epidemiological features of NBD are various. In our Tunisian cohort of NBD patients the main characteristic features were male predominance, a relatively high prevalence of CVT, a low prevalence of intra-cranial hypertension and a significant lower frequency of HLA-B51 haplotype.

[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.

Association of right heart thrombosis, endomyocardial fibrosis, and pulmonary artery aneurysm in Behçet's disease.

A 29-year-old man with a 5-year history of Behçet's disease was admitted for fever, dyspnae, chest pain, and hemoptysis. A diagnosis of right ventricle and atrial thrombosis associated with a pulmonary artery aneurysm was made. The patient was treated with anticoagulants and prednisone. Since hemoptysis persisted, surgical excision of the intracardiac thrombosis was performed and histological findings were consistent with organizing thrombus and endomyocardial fibrosis. Transesophageal echocardiography 6 months later showed recurrence of the right ventricle thrombosis. A course of 6-monthly boluses of intravenous cyclophosphamide was begun. Currently, at 2 years of follow-up, the patient is asymptomatic.