Uncovering the Spectrum of Hemophagocytic Lymphohistiocytosis: A Nephrology Department's Analysis of 14 Cases.

Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a disease of multi-organ dysfunction due to excessive immune activation causing widespread inflammation and tissue destruction. It is a severe condition associated with high morbidity and mortality. Early identification is crucial for prompt treatment. The objective of this case series is to underscore the intricacy of managing HLH in individuals with renal dysfunction. Methods: This is a retrospective study of patients diagnosed with HLH in a nephrology department over a period of 30 years. We retrospectively reviewed the medical files by applying the Revised HLH-2004 criteria. Results: Among the 14 female patients included, the mean age was 45.2 years (range 23-78). Nine patients presented with sudden onset of fever and chills. Physical examination revealed purpura in 3 cases, hepatomegaly and splenomegaly in 6 and 5 cases respectively, and peripheral lymphadenopathy in 1 case. Hemorrhagic complications were observed in 5 cases, hypertriglyceridemia in 9 cases, and hyperferritinemia in all cases. Hypothyroidism was observed in all cases, and impaired renal function was detected in 11 of them, with 5 experiencing it as a result of lupus nephritis, and 1 case attributed to pre-eclampsia. Hemophagocytosis was confirmed through sternal puncture in 11 cases. Treatment involved etiological therapy with corticosteroids and immunosuppressants and/or anti-infectives. Intravenous immunoglobulins were administered in 6 cases, while 2 cases required coagulation factor transfusions. Unfortunately, 9 patients did not survive. Conclusion: The study highlights the need for increased awareness and prompt recognition of HLH, particularly in patients with associated renal complications.

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.

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.

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.

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.

Chronic inflammatory rheumatism associated with Takayasu disease.

Takayasu disease is rarely associated with other autoimmune diseases. Therefore, the cases discussued herein are uncommon because we are reporting Takayasu disease associated with rheumatoid polyarthritis and spondylarthropathy. The first case concerns a 40-year-old woman presenting with Takayasu disease 11 years after the diagnosis of erosive and seronegative rheumatoid polyarthritis. The upper limb arteries and 1 lower limb artery were affected. The second 41-year-old case presented with ankylosing spondylitis that had been evolving for 10 years. Human leukocyte antigen-B27 typing was negative. Takayasu disease was revealed by severe high blood pressure. In both cases, radiologic examination revealed a typical aspect of the aorta and its main collaterals. Rarely in the literature have these associations been reported, and the pathology remains unknown.

[Etiologic profile of amyloidosis of the elderly in Tunisia]. / Profil étiologique de l'amylose en Tunisie chez les sujets âgés.

BACKGROUND: The amyloidosis is one of the histologic lesions the most frequently associated to the aging. AIM: To identify the several etiologies of amyloidosis in elderly patients according to immunohistochemical type. METHODS: Retrospective study on 10 years, in Internal medicine and Nephrology department in Charles Nicolle Hospital. Tunis. Tunisia. Diagnosis of amyloidosis was retained after histological confirmation with specific colorations, in patients aged 65 years or more at the diagnose of amyloidosis. RESULTS: The study enrolled 51 patients with amyloidosis. In 67% of cases it was AA amyloidosis. The etiology the most frequently observed with this type was the tuberculosis, followed by chronic pulmonary infections. We retained diagnose of non AA amyloidosis in 21% of patients. The multiple myeloma has been the most frequent etiology observed, but no cause was identified at about half of patients. Twelve per cent of amyloidosis were not typed. CONCLUSION: From our study, we can remark the high predominance of AA amyloidosis, probably because of frequency of tuberculosis which is still high in our country, this is not in accordance with occidental data, were AL amyloidosis and senile amyloidosis predominate in elderly.

[Renal involvement in Behcet's disease]. / L'Atteinte Renale au cours de la Maladie de Behçet.

BACKGROUND: Renal involvement in the Behcet's disease is rare. The clinical features vary from urinary sediment's abnormalities to ESRD. AIM: We propose to study the clinical, biological and histological data, the therapeutic management and the prognosis of patients. METHODS: We report a retrospective study including 8 patients representing 1.23 % of cases. RESULTS: The average age of the patient was of 37 +/- 12. 35 years with a clear male prevalence. Urinary signs were discovered fortuitously by the strips in the majority of the cases after an average of 18 months. It's about proteinuria and hematuria. Renal insufficiency and hypertension were rare. Pathological study highlighted 3 cases of amyloidosis, 2 cases of IgA nephropathy, 1 case of minimal change disease, 1 case of endo and extracapillary glomerulonephritis and 1 case of interstitial nephropathy. Patients having GN were treated by corticoids and immunosuppressive agents and those having an interstitial nephropathy were treated symptomatically with good evolution in the majority of the cases. Only one patient is dead, he had amyloidosis. Prognosis depended on the precocity of the diagnosis, the histological type and the treatment. CONCLUSION: The renal involvement during Behçet's disease is rare. Amyloidosis and Ig A nephropathy are the most frequent. Treatment is still controversial.