Tuberculosis and Takayasu arteritis: a case report.

BACKGROUND: Takayasu arteritis is a rare and chronic granulomatous vasculitis that affects the large vessels. Takayasu arteritis targets the aorta and its branches and is still of unknown etiology. It often affects female patients under 50 years of age. A relationship between Takayasu arteritis and tuberculosis has been suggested for a long time. CASE PRESENTATION: We report a severe case of Takayasu arteritis in a 10-year-old Tunisian child revealed by renovascular hypertension with concomitant pulmonary tuberculosis. CONCLUSIONS: Our patient is among only a few cases of Takayasu arteritis published worldwide affecting young infants and adolescents, which underlines the strong relationship between Takayasu arteritis and tuberculosis.

Fulminant lupus pneumonitis complicating systemic lupus erythematosus in the elderly.

Fulminant lupus pneumonitis is a rare complication of SLE. We report a case of 75 years-old male patient with SLE who developed pneumonia and severe respiratory failure requiring mechanical ventilation. Refractory respiratory distress complicating noninfectious fulminant lupus pneumonitis did not respond to methylprednisolone and intravenous immunoglobulin treatment.

Tuberculous tenosynovitis of the hand: A challenging diagnosis.

Tuberculous tenosynovitis of the hand is a very rare condition. Herein, we report the case of tuberculous tenosynovitis of the hand in a 32-year-old woman. We highlight the success of anti tuberculosis drugs alone without resort to surgical treatment.

Health related quality-of-life in systemic sarcoidosis in Tunisia.

INTRODUCTION: Sarcoidosis is a systemic granulomatosis that can be associated with large-scale physical and mental disability, affecting the health related quality-of-life (HRQoL) of patients. AIM: To evaluate the HRQoL of tunisian patients with sarcoidosis and to identify the factors that influence it. METHODS: We conducted an analytical, cross-sectional study collecting 31 patients with sarcoidosis according to the ATS/ERS/WASOG criteria. The evaluation of the HRQoL was assessed by two questionnaires in tunisian dialect. The generic score was the Medical Outcome Study 36-Short Form Health Survey (SF-36).The specific score used was the Sarcoidosis Health Questionnaire (SHQ). RESULTS: The HRQoL of our 31 patients was more affected in the three domains of the SHQ compared to the SF-36, which is in favor of the better sensitivity of the SHQ to detect the influence of the extent of sarcoidosis on the HRQoL. Factors associated with more impaired HRQoLwere: age at disease onset, age at interview, comorbidities, altered spirometry results, ocular involvement, chronic cholestasis, splenic nodules, arthralgia, organ count ≥3, lymphopenia and cholestasis at the time of the interview. Taking an immunosuppressant agent, particularly Methotrexate, was associated with HRQoL improvement. The number of relapses was the most correlated factor with an altered HRQoL, and this in several domains. CONCLUSION: For an effective management of patients with sarcoidosis, a bio-psycho-social approach is now necessary in order to assess the real and global impact of the disease and to improve the HRQoL of patients. Disease-specific scores seem more reliable in achieving these goals.

[Isolated laryngeal tuberculosis as an uncommon manifestation of extrapulmonary tuberculosis in adults: a case report]. / Tuberculose laryngée isolée de l'adulte: localisation extra pulmonaire exceptionnelle (à propos d'un cas).

Isolated laryngeal tuberculosis is rare and sometimes difficult to diagnose. It is the most common cause of laryngeal granuloma. We here report the case of a 58-year-old man, with no particular past medical history, hospitalized due to paroxysmal laryngeal dyspnea, dysphagia to solid foods and dysphonia evolving for 6 months without other associated signs. Laryngoscopic examination showed polyploid formation masking the glottic floor. Histological examination revealed epithelioid and gigantocellular granuloma, without caseous necrosis. Direct microscopic examination and culture were negative. The diagnosis of isolated laryngeal tuberculosis was made based on the endemicity in our country and the absence of other arguments in favor of another type of granulomatosis. Anti-tuberculosis therapy, combined with oral corticosteroids, was indicated based on the presence of severe upper airway edema and symptoms were resolved after 40 days of treatment.

Early onset of scleromyxedema Arndt-Gottron associated with a monoclonal gammapathy: Successful treatment with intravenous immunoglobulins.

Arndt-Gottron (S-AG) syndrome or scleromyxedema is a scarce disease characterized by a generalized papular and sclerodermoid eruption and systemic manifestations that can lead to significant morbid-mortality. Interesting, S-AG can be associated with a paraprotein. We report an exceptional scleromyxedema case associated with a benign monoclonal gammapathy in an old woman.

Renal Amyloidosis: Epidemiological, Clinical, and Laboratory Profile in Adults from One Nephrology Center.

Background: Renal amyloidosis is one of the main differential diagnoses of nephrotic proteinuria in adults and the elderly. The aim of this study with the most important series in our country is to contribute to the epidemiological, clinical, and etiological study of the renal amyloidosis. Methods: In a retrospective study carried out between 1975 and 2019, 310 cases of histologically proven and typed renal amyloidosis were selected for this study. Results: There were 209 men and 101 women with a mean age of 53.8 ± 15.4 years (range, 17-84 years). Of the 310 cases, 255 (82.3%) were diagnosed with AA renal amyloidosis and 55 (17.7%) with non-AA amyloidosis. Infections were the main cause of AA amyloidosis, and tuberculosis was the most frequent etiology. The period from the onset of the underlying disease to diagnosis of the renal amyloidosis was an average of 177 months. The most frequent manifestations at the time of diagnosis were nephrotic syndrome (84%), chronic renal failure (30.3%), and end-stage renal disease (37.8%). After a medium follow-up of 16 months (range, 0-68 months), mortality occurred in 60 cases. Conclusions: Given the high frequency of AA amyloidosis in our country, awareness of the proper management of infectious and chronic inflammatory diseases remains a priority in reducing the occurrence of this serious disease.

Systemic lupus erythematosus-related acute pancreatitis: An exceptional form with severe exocrine and endocrine pancreatitic failure in a Tunisian child.

Acute pancreatitis may be the first manifestation in systemic lupus erythematosus or occur during evolution. It is a rare complication, which is often associated with other visceral manifestations. Outcome is usually favorable but can be serious. We report a case of a 17-year-old girl with a past history of systemic lupus erythematosus who developed acute pancreatitis revealed by abdominal pain. Elevated serum amylase and lipase levels and pancreatic enlargement on tomography confirmed the diagnosis. Although high-dose corticosteroid was prescribed, the patient died from a refractory diabetic ketoacidosis.

Acquired Bartter Syndrome in Primary Sjögren Syndrome.

Renal tubular involvement in Sjögren's syndrome (SS) often described with renal tubular acidosis, nephrogenic diabetes insipidus, or rarely with Fanconi syndrome. SS presenting with clinical features of Bartter's syndrome or Gitelman's syndrome is rare. We report a case of a female patient who presented an acquired Bartter syndrome with a primary SS.

Extensive sphenoid chordoma mimicking a prolactinoma.

The chordoma is a benign cartilaginous tumor whose sphenoidale localization is exceptional. This tumor has considerable difficulties of both diagnosis and treatment. We report the observation of a Tunisian adult who presented features of hypopituitarism set wrongly on account of a prolactinoma.

Prolonged fever and intra-cardiac thrombosis revealing Behçet's disease.

BACKGROUND: Prolonged fever (PF) is a challenging problem for physicians since it can be the first manifestation of a large variety of pathologies. Exceptionally, intra-cardiac thrombus (ICT) could explain PF and reveal Behçet's disease (BD). We are reporting a 45-year-old man with BD who has these unusual manifestations. CASE REPORT: A 45-year-old man presented with PF and inflammatory biological syndrome during a few months. Echocardiography showed an inhomogeneous and mobile mass in the left auricle. During his hospitalization, the patient had multiple oral aphtosis. The angioscanner showed a sacciform aneurysm of the coeliac trunk. The diagnosis of BD was retained and he was treated with high doses of steroids and cyclophosphamide with a favorable follow-up. CONCLUSION: The search for BD should be systematic in view of any suggestive manifestation of severe cardiovascular complications like ICT especially in a young adult man from a high endemicity region.

Successful resolution of Hemophagocytic lymphohistiocytosis associated to brucellosis in the adult.

Hemophagocyticlymphohistocytosis (HLH) is a proliferation of histiocytes with importanthemophagocytosisoccurring in different organs such as the spleen and the bone marrow. HLH is now increasingly diagnosed in the context of infections, malignancies and connective tissue diseases. Although brucellosis is an endemic infection in Tunisia, its association with HLH is a very rare condition which should be considered in patients with splenomegaly and cytopenia. Here, we describe brucellosis associated HLH in a 31 year-old man. The patient was admitted to our hospital with fever, sweating, and fatigue. Physical and laboratory findings revealed splenomegaly, pancytopenia, elevated serum transaminases, triglycerides, lactate dehydrogenase, and ferritin, and bone marrow hemophagocytosis. The Brucella agglutination test was positive. The patient improved after treatment with Rifampin and doxycyclin.

[Renal prognosis of IgA vasculitis nephritis in adult patients: a monocentric study of 25 cases]. / Pronostic rénal de la néphropathie des vascularites à IgA de l'adulte: étude monocentrique, à propos de 25 cas.

IgA vasculitis nephritis affects the prognosis of this disease in adult patients. This study aimed to examine the clinical characteristics of this renal involvement in adults and to identify factors influencing renal prognosis. We conducted a retrospective monocentric study of patients with histologically confirmed IgA vasculitis nephritis (rheumatoid purpura) (EULAR classification criteria) with renal involvement classified according to Pillebout classification. We analyzed renal survival and identified the factors influencing renal prognosis. Twenty-five patients were included (sex ratio M/F = 2.57), their average age at diagnosis of rheumatoid purpura was 35,76 years. Purpura was diagnosed in 100% of cases, with articular involvement in 28% of cases. Renal failure was identified in 44% of cases. The most common histological classification was IgA vasculitis nephritis (class II). Clinical remission was observed in 44% of cases and an evolution toward chronic renal failure (end-stage renal disease) in 36% of cases. Renal survival at 195 months was 57%. The identified prognostic factors were digestive involvement (p = 0.022), early renal failure (p = 0.0004), glomerular classification (P=0,001) and the severity of the histological lesions, renin-angiotensin system blocker treatment (p = 0.01) and plasma exchanges (p = 0.03). Our study shows that renal involvement during IgA vasculites can be relatively severe with poor renal prognosis. The identification of clinical and histological prognostic factors may be useful as guidance for the development of prospective therapeutic studies.

A Case of Type 1 Autoimmune Pancreatitis (AIP), a Form of IgG4-Related Disease (IgG4-RD).

BACKGROUND Type 1 autoimmune pancreatitis (AIP), also known as lymphoplasmacytic sclerosing pancreatitis (LPSP), is a rare cause of chronic pancreatitis, characterized by a fibro-inflammatory process. However, patients with AIP may have a good response to corticosteroid therapy. We describe a Tunisian patient with AIP that was confirmed to be an IgG4-related disease (IgG4-RD). CASE REPORT We describe a case of a 70-year-old man who was admitted to hospital for obstructive jaundice and abdominal pain. Serum liver function tests were abnormal and upper abdominal computed tomography (CT) imaging showed diffuse pancreatic swelling and strictures of the main pancreatic duct without any focal lesion. Pancreatico-biliary magnetic resonance imaging (MRI) showed a thickened rim surrounding the pancreatic duct Serum IgG4 levels were elevated, resulting in a diagnosis of IgG4-related AIP. The patient showed a good clinical, biochemical, and radiological response following steroid therapy in combination with azathioprine. CONCLUSIONS The diagnostic workup of IgG4-RD is complex and usually requires a combination of clinical examination, imaging, and serological analysis. As this case report has demonstrated, IgG4-RD should be considered in patients who present with pancreatitis or AIP, because of the favorable response to steroid therapy, particularly when treatment is initiated early.

A severe anti MDA-5 antibodies associated amyopathic dermatomyositis.

BACKGROUND: Lung damage during amyopathic dermatomyositis (ADM) associated with auto antibodies anti MDA-5 is serious. We report a rare observation of a severe ADM associated to anti MDA-5 antibodies complicated with diffuse interstitial lung disease (ILD) rapidly extensive and fatal. OBSERVATION: We report the observation of a Tunisian adult who was admitted for ADM. The anti MDA-5 antibodies were detected. He developed an ILD rapidly extensive to fibrosis. Corticosteroids were unefficient and he deceded of pneumomediastin and refractory respiratory distress. CONCLUSION: This observation illustrates the severity of ADM with anti MDA-5 antibodies. Every clinician should detect any ILD to avoid the progression to fibrosis.

[Celiac disease in adult patients revealed by polyserositis: about a case]. / Maladie cœliaque de l'adulte révélée par une polysérite.

Celiac disease (CD) is an autoimmune disease affecting multiple organs. It often presents as gastrointestinal manifestations associated with malabsorption. However, serosa involvement uncommonly reveals this enteropathy, making the diagnosis difficult. We here report the case of JA, aged 63 years, admitted to hospital to detect the cause of malabsorption syndrome associated with polyserositis signs including pleurisy, pericarditis, ascites and hydrocephalus. The diagnosis of CD was based on endoscopic signs without serology tests. Patient's evolution was partially favorable, due to lack of compliance with a gluten-free diet. Our study reports the first case of CD revealed by polyserositis. CD should be suspected in patients with malabsorption syndrome, in the absence of evocative signs.

[Pseudotumor cerebri revealing sarcoidosis]. / Pseudotumeur cérébrale révélant une sarcoïdose.

Sarcoidosis is a multivisceral granulomatosis of unknown aetiology which may have various clinical and radiological manifestations. Cerebral sarcoidosis, although rare, can appears as a misleading pseudotumor. We report the case of a young Tunisian adult hospitalized for intracranial hypertension associated with pseudotumoral lesion on radiology, which was revealed to be systemic sarcoidosis.