Compared with the general rheumatology clinic, the dedicated lupus clinic had better quality measure performance in this cross-sectional single-center study. In our health care system, we also observed indicators suggesting that rheumatologists with a higher volume of SLE patients provide higher quality of care.
Biliary sump syndrome is a rare condition. It is seen as a rare long-term complication in patients with a history of a side-to-side choledochoduodenostomy. In the era before endoscopic retrograde cholangiopancreatography, side-to-side choledochoduodenostomy was a common surgical procedure for the management of biliary obstruction. In the setting of a side-to-side choledochoduodenostomy, the bile does not drain through the distal common bile duct anymore. Therefore, the part of the common bile duct distal from the choledochoduodenostomy anastomosis consequently transforms into a poorly drained reservoir, making this so-called “sump” prone to accumulation of debris. These patients are prone to cholangitis. We present a 64-year-old man with a history of side-to-side choledochoduodenostomy who presented with manifestations of cholangitis. An endoscopic retrograde cholangiopancreatography confirmed a diagnosis of sump syndrome. The etiology, clinical manifestations, and treatment of biliary sump syndrome are discussed in this article.
Patient: Female, 40Final Diagnosis: Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)Symptoms: Gait disorderMedication: —Clinical Procedure: —Specialty: RheumatologyObjective:Rare diseaseBackground:Chronic inflammatory demyelinating polyneuropathy (CIDP) is an uncommon manifestation of systemic lupus erythematosus (SLE). We report a case of SLE presenting as CIDP and discuss the diagnosis, management, and prognosis of CIDP.Case Report:A 40-year-old woman with a past medical history of SLE treated with hydroxychloroquine presented with bilateral, progressive, ascending, sensory and motor neuropathy. Physical examination showed weakness and reduced temperature of all extremities, reduced pinprick and vibration sense of the distal extremities, loss of reflexes, and walking with a wide-based unsteady gait. Laboratory investigations showed positive antinuclear antibodies (ANA), anti-(smooth muscle (SM) antibody, anti-RNP antibody, anti-SSA antibody, anti-ds-DNA antibody, and an erythrocyte sedimentation rate (ESR) of 75 mm/hr, low C4, leukopenia, and anemia. Electromyography (EMG) confirmed the diagnosis of CIDP. The patient’s neuropathy and muscle weakness improved on treatment with intravenous immunoglobulin (IVIG) and high-dose steroids.Conclusions:The early clinical diagnosis of CIDP, supported by serological autoantibody profiles associated with SLE, can predict a good response to steroids. Most patients with CIDP are treated successfully with steroids if the diagnosis is made early. IVIG, plasmapheresis, or immunosuppressive therapy should be considered if there is no response to steroids.
Staphylococcus lugdunensis is a coagulase-negative staphylococcus, which in humans can cause virulent infections frequently attributable to Staphylococcus aureus including superficial skin/soft tissue infections, infective endocarditis, osteomyelitis and endophthalmitis. A 61-year-old male with history of recent fixation of C1-C2 neck fracture, presented with sudden onset of right-sided weakness and fever. Examination revealed dense hemiplegia with UMN facial palsy of right side, and a pan-systolic murmur in the mitral area, radiating to the axilla. He had an elevated troponin-I of 18.8 ng/mL with right bundle branch block on EKG. Echocardiogram revealed mobile vegetation on the anterior leaflet of the mitral valve with ejection fraction of 55% and no regional wall motion abnormality. He continued to have persistent high-grade fevers in spite of appropriate antibiotic therapy and blood cultures were persistently positive for S. lugdunensis for 5 days. He developed weakness of his left side after 2 weeks of hospital admission and MRA revealed left carotid dissection and complete occlusion of the right middle cerebral artery distal to the M1 segment which was embolic in nature. The frequency of S. lugdunensis infection is underappreciated and biofilm formation plays a major role in the pathogenesis. This is the first reported case of S. lugdunensis endocarditis complicated by NSTEMI from coronary embolism and simultaneous stroke from cerebral embolism. Due to the early, frequent and fatal complications from S. lugdunensis bacteremia, we might have to consider early surgical intervention in the absence of class I or IIa recommendation.
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