ABSTRACT
Introduction: Systemic lupus erythematosus (SLE) is a chronic, multifaceted autoimmune inflammatory disease with a wide range of clinical presentations resulting from its effect on multiple organ systems. We report a case of SLE associated with autoimmune pancreatitis. Methods: In this study, we present a patient diagnosed as having SLE who developed acute auto-immune pancreatitis. Results: This is a 36-year-old woman, with lupus diagnosed since 2009. Initially, the manifestations of her disease were dermatological and articular. Then appeared the renal involvement with a lupus nephropathy class IV at the renal biopsy (PBR). She was previously treated with the NIH protocol then oral prednisolone with improvement in her symptoms. She continued these medications but was lost to follow-up since 2016 and presented after 6 years with pigmented skin lesions on her upper and lower limbs, abdominal pain and distension, vomiting, and an altered general condition. In biology, the patient presented a functional acute kidney failure, an elevated amylasemia (30 times normal), an elevated lipasemia (6 times normal), a normocytic normochromic hemolytic anemia with positive direct coombs test, lymphopenia, a positive immunological assessment (AAN, anti DNA AC, anti Sm, anti SSA, anti RNP), a low C3, a low C4. The patient presented a lupus flare with a SLEDAI score of 6 points: moderate lupus activity. Ultrasound confirmed a large abundance of ascites. Ascites fluid puncture showed an exudate with hyperleukocytosis with predominantly PNN and no germ on direct examination nor on culture.The infectious origin of the pancreatitis was eliminated (CMV, tuberculosis, covid19), as well as the tumoral origin (negative tumor markers, abdominal CT scan showed a swollen pancreas in its caudal portion with loss of physiological lobulations and normal spontaneous density.Necrosis flows difficult to individualize. In addition, no deep neoplastic focus). The autoimmune origin of the pancreatitis due to its lupus attack was retained. She was put on corticosteroids (500mg intravenously for 3 days) then relayed by oral route, albumin infusion, evacuation puncture. The subsequent evolution was marked by the progressive normalization of the pancreatic balance and the slower disappearance of the ascites. Conclusions: Acute pancreatitis is an unusual manifestation of SLE and it should be suspected in any SLE patient with these similar symptoms. In many cases, this complication has been attributed to the drugs administered. In our case, a favorable course of pancreatitis with corticosteroids adds further evidence to the idea that lupus-related pancreatitis is not a side effect of corticosteroid therapy. Moreover, treatment with these medications improves the prognosis. No conflict of interest
ABSTRACT
Introduction: Crohn's disease (CD), a polygenic disorder, is the major subtype of inflammatory bowel disease. There is disease heterogeneity and many patients present extraintestinal manifestations. However, the association of crohn's disease with another genetic disease has not been previously described. Methods: We report the exceptional association of CD with Glanzmann’s thrombasthenia which is a rare inherited bleeding disorder. Indeed, the diagnosis in a patient of kidney amylosis, which we think secondary to CD, has revealed the association of the two diseases in a Tunisian family. Results: A 38 years old man was admitted in August 2020 in our Nephrology department for management of severe nephrotic syndrome with normal renal function. He was born of first-cousin consanguineous parents and diagnosed with Glanzmann's thrombasthenia since childhood. His family history was remarkable: His mother and maternal uncle had CD, his sister have also Glanzmann's thrombasthenia. At examination,blood pressure was 09/60 mmHg and the patient was in anasarque and complained of chronic diarrhea. Given renal biopsy was a high bleeding risk procedure in this patient with Glanzmann thrombasthenia, we decided to perform biopsy of salivary gland and Congo red staining showed the presence of characteristic amyloid deposits. During hospitalization the patient presented persistent diarrhea without fever. Enteroscann showed prominent wall thickening and mucosal hyperemia encompassing a 10 cm segment of ileum. Colonoscopy found discontinuous distribution of longitudinal ulcers with small aphthous ulcerations typical of CD. Biopsy was not performed because the risk of bleeding was high. The diagnosis of renal amyloidosis as a complication of CD was made. Treatment was symptomatic with the introduction of systemic corticosteroid therapy as a treatment for CD. Evolution was marked by the occurrence of acute kidney failure in conjunction with covid 19 infection. Conclusions: We reported the uncommon association of Glanzmann’s thrombasthenia with renal amyloidosis complicating CD in a patient with remarkable family history of consanguineous marriage. It is an association of two hereditary diseases never reported in literature. We believe that a genetic explanation must certainly exist. The family investigation is ongoing. No conflict of interest
ABSTRACT
Introduction: Hemodialysis emergencies in nephrology are frequent. Prompt diagnosis and management are needed to save life and enhance prognosis. The aim of this work was to determine the hemodialysis’ prevalence in emergency situations, its indications, the clinical, paraclinical and evolutionary profiles and to describe the parameters of the session as well as the per-dialytic incidents and their impacts on patient mortality. Methods: It is a retrospective monocentric descriptive study during 3 months (July, August, and September 2020) carried out in the nephrology department of the University teaching hospital of Sousse. We collect patients who underwent at least one session of HD. Results: We included 61 patients who underwent emergency hemodialysis. The average age of our patients was 58±17 years (17- 95) and the sex ratio was 1,5. Our patients have hypertension and diabetes in respectively 56,1% and 34,4% of cases. 42,6% of patients were admitted from the emergency department, 27,9% of patients from the surgical and urology department, 18% of patients from nephrology departments and 11,5% of patients from other departments. Our population consists in chronic HD patients in 34,5%. Chronic renal failure was noted in 43,6% with diabetic nephropathy as the most primary cause (44,1%). Acute renal failure was found in 18.2% with Obstructive nephropathy (66,1%) as the main etiology.two patients were transplanted (3,3%). HD indications were hyperkalemia in 30%, acute pulmonary edema in 25% and metabolic acidosis in 23,3% of cases. Conventional HD was performed with mean session duration of 3,4 hours. Ultrafiltration was required in 73,8% with an average of 1800ml (range: 500-4000).The most frequently used approach was the femoral route in 54,1% followed by arterioveinous fistula in 24,6%. Transfusion was required in 26,2% of cases. Perdialytic complications were observed in 29,5% of cases. Hypotension was the most common complication present in 13,1% of case;which leads to the interruption of the session in 4.9% of cases;followed by agitation in 4,9% of case. The renal evolution of our population was hemodialysis dependence in 35,6%, back to basic level in13,3%chronic kidney disease in4,4% and recovery in 2,2%. Death occurred in 5 cases including 3 patients outside dialysis and 2 patients on dialysis. Infection by SARS covid 19 was the cause of death in 1 patient. Conclusions: Extra-renal purification (REE) is sometimes essential in an emergency situation in the management of severe ARI or terminal CKD. Early diagnosis and relevant treatment will enhance the prognosis of patients in the short and medium term. No conflict of interest