Primary membranous nephritis (PMN) is an autoimmune disease induced by the deposit of antibodies (Ab) to the phospholipase receptor A2 receptor (PLA2R) on podocytes. In this context, we aimed to ...assess the relationships between anti-PLA2R Ab, PLA2R rs4664308 SNP, PLA2R mRNA levels and PMN susceptibility and outcome. Sixty-eight PMN patients, 30 systemic lupus erythematosus (SLE) patients with secondary MN and 30 healthy control subjects served for anti-PLA2R Ab measurement by ELISA and PLA2R rs4664308 SNP genotyping by a commercial real-time PCR. Twenty patients with tubulo-interstitial nephritis (TIN) were used as controls for renal PLA2R mRNA quantification in PMN patients from kidney biopsies. PLA2R mRNA quantification was carried-out by real-time PCR after RNA extraction. Forty-three (63.2%) PMN patients received initial therapy consisting of alternating monthly cycles of corticosteroids and cyclophosphamide. Twelve (17.6%) patients had resistant PMN to initial therapy and were consecutively treated by cyclosporine or tacrolimus. Anti-PLA2R Ab and renal PLA2R mRNA could be useful markers for PMN outcome predicting. The PLA2R rs6446308 SNP is associated with PMN susceptibility in Tunisians.
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.
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.
SLE‐related AP is a rare and severe condition. Diagnosis is based on amylasemia and abdominal scanner. Each practitioner has to focus on endocrine pancreatic failure which is severe and life‐threatening.
Thalidomide, which is an angiogenesis inhibitor and immunomodulator that reduces tumor necrosis factor-alpha, has regained value in the treatment of multiple myeloma. Serious pulmonary complications ...due to thalidomide use remain relatively uncommon. We describe a case of bronchiolitis obliterans organizing pneumonia (BOOP) due to thalidomide. A 51-year-old man with IgG lambda myeloma was treated with thalidomide and dexamethasone. Seven days after the beginning of chemotherapy, the patient presented a fever and a persistent cough. Auscultation revealed crackles in both pulmonary bases. The chest X-ray showed a diffuse bilateral alveolar-interstitial syndrome. Computed tomography scan revealed bilateral pulmonary involvement, with bilateral interstitial alveolar infiltration and ground-glass pattern consolidations. Pulmonary infection, malignant tumor, and lung involvement of multiple myeloma were excluded through various tests. Thalidomide-induced BOOP was suspected, and the drug was withdrawn and replaced by Melphalan. The patient had complete resolution of his symptoms and radiologic pulmonary involvement on discontinuation of the drug. In the absence of other etiologies, physicians should be cognizant of this potential complication in patients receiving thalidomide who present with respiratory symptoms.
Autosomal recessive polycystic kidney disease (ARPKD) is the most common childhood-onset ciliopathy. Intracranial aneurysms (ICA) are a serious complication of autosomal dominant polycystic kidney ...disease (ADPKD). However, there are only three reports about ICA in an adult patient with ARPKD. We describe a rare case of a 29-year-old man with ARPKD presenting with subarachnoid hemorrhage secondary to a ruptured intracranial aneurysm. The diagnosis of ARPKD was at the age of eight years based on typical ultrasonography findings with polycystic kidneys and liver disease. Magnetic resonance cholangiography showed a nonobstructive dilatation of intrahepatic bile ducts. Liver biopsy showed hepatic fibrosis. None of the family members was affected. At the age of 15 years, he had progressed to end-stage kidney disease, and hemodialysis was started. The patient had always a severe arterial hypertension. At the age of 29 years, he complained of headaches with an uncontrolled hypertension and disturbance of consciousness, computed tomography angiography showed subarachnoid hemorrhage and multiple cerebral aneurysms. Early neurologic screening of intracranial aneurysm should be recommended in ARPKD like in ADPKD patients.
We report clinical and renal histological data, treatment modalities and outcome of 43 Tunisian children with biopsy-proven lupus nephritis seen over a 23-year period. There were 39 girls and 4 boys ...with a mean age of 12.5 years at diagnosis of lupus nephritis and followed for a mean period of 77 months. Renal symptoms included urinary abnormalities in all patients, hypertension in 40% of cases, nephrotic syndrome in 60% of cases and renal failure in 25% of cases. Class IV and class III nephritis were observed in 48.8 % and 30.2 % respectively. Corticosteroids were used in all cases, associated to immunosuppressive therapy in 23%. Overall survival was 86% at 5 years and 74% at 10 and 15 years. Renal survival was 83% at 5 and 10 years and 63% at 15 years. Initial renal failure and tubulointerstitial fibrosis were significantly increased risk for the development of end-stage renal disease in our study group. Renal histological findings provide the basis for treatment recommendations. Timely performed renal biopsy is greatly needed to accurately determine the prognosis and to guide treatment in children lupus nephritis.