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.
FLP in SLE of the elderly is a rare and severe condition. Each practitioner has to focus on respiratory failure, which is severe and life‐threatening.
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.
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.
T1 weighted MRI images showed thickening of flexor sheaths (A), intensely enhanced after injection of gadolinium (B), conforming with flexor tenosynovitis of the left hand.
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.
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.
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.
La sarcoïdose est une granulomatose multi viscérale d'étiologie inconnue qui peut revêtir des tableaux cliniques et radiologiques diverses. Les localisations cérébrales bien que rares, peuvent se ...présenter sous forme pseudo-tumorale trompeuse. Nous rapportons l'observation d'un jeune adulte Tunisien hospitalisé pour hypertension intracrânienne en rapport avec une lésion pseudotumorale radiologique qui a révélé une sarcoïdose systémique.
Disturbed kidney function is a common complication after bone marrow transplantation. Recently, attention has been given to immune-mediated glomerular damage related to graft versus host disease ...(GVHD). We describe a 19-year-old woman who developed membranous glomerulonephritis after bone marrow transplantation (BMT). Six months later, she developed soft palate, skin and liver lesions considered to be chronic GVHD. Fifteen months after undergoing BMT, this patient presented with nephrotic syndrome. A renal biopsy showed membranous glomerulonephritis associated with a focal segmental glomerulosclerosis. She was started on corticosteroid treatment with good outcome.
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.
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