Systemic lupus erythematosus presenting as hemolytic uremic syndrome: a case report

Associating systemic lupus erythematosus (SLE), with an initial presentation of hemolytic uremic syndrome (HUS) is rare. We report a case of 21-year old Afghani female admitted to our hospital with an initial complaint of high grade fever and diffuse maculopapular rash and swelling of lower limbs. Diagnosis of atypical HUS was established according to the clinical triad of HUS without a veriotoxin-producing organism in her stool and the pathological finding compatible to thrombotic microangiopathy. In addition, her symptoms fulfilled the 1982 revised criteria for the classification of SLE. After pulse methylprednisolone, cyclophosphamide and plasmapheresis therapies, her laboratory findings and general condition improved. Unfortunately she was lost to follow up as she decided to return back to Afghanistan

Platypnea-orthodeoxia: report of two cases and review of the literature

We describe two unusual cases of platypnea. The first patient had chronic obstructive pulmonary disease, but platypnea did not respond to chronic obstructive pulmonary disease therapy. He was found to have multiple pulmonary emboli, and symptoms rapidly improved on anticoagulation therapy. The second patient had Parkinson disease and developed severe platypnea, an association that has not been previously described. She had significant postural hypotension and responded to therapy with fludrocortisone

Association of angiotensin-converting enzyme gene dimorphisms with severity of lupus disease.

Angiotensin-converting enzyme (ACE) plays an important role in the development of systemic lupus erythematosus (SLE) because its end-product, angiotensin II, plays an integral role in the regulatory system responsible for endothelial control and vascular tone, systems that are commonly affected in Patients with SLE. Additionally, ACE inhibitors have been shown to retard the progression of SLE and lupus nephritis. Our goal was to investigate whether ACE gene polymorphisms are associated with increasing severity of SLE. We genotyped 39 SLE Patients of varying disease severity from a homogenous Asian population and 79 control subjects for ACE I/D and 2350 G \u3e A dimorphisms. All Patients met the American College of Rheumatology (ACR) criteria for SLE and their disease severity was measured using Systemic Lupus Activity Measure (SLAM). The A allele was found to be associated with increase in severity of SLE with the AA genotype present only in severe disease. No association with SLE in general, compared to healthy subjects, was found with either dimorphism. We also examined the transmission of haplotypes as defined by these polymorphisms. The D and A alleles were found in strong linkage disequilibrium especially in severe SLE. The DA-haplotype was more frequent in severe SLE, than mild to moderate disease. Our findings suggest that DNA sequence variation in the ACE gene influences disease progression and severity of SLE

Herpes simplex encephalitis: analysis of 68 cases from a tertiary care hospital in Karachi, Pakistan

Objective: To evaluate clinical presentation, radiological and cerebrospinal fluid findings and outcome of patients with Herpes simplex encephalitis (HSE). Methods: The charts of all the patients (n = 88), who were admitted to The Aga Khan University, Karachi with diagnosis of HSE, from 1990-2002, were retrospectively reviewed. Sixty eight patients were included in the study. The variables were identified (including demographic data, signs and symptoms at presentation and laboratory investigations such as CSF analysis including, PCR, serum IgM antibodies, EEG and neuroimaging). The patients were included in the study if they had any three of the five criteria positive in addition to clinical features suggestive of herpes encephalitis. Results: Sixty eight patients, that fulfilled the criteria, were included in the study. Clinical findings included fever, seizures, altered mental status, aphasia and hemiparesis. CSF, analysed in all the patients, was abnormal in 65 patients (96%) and EEG was abnormal in 82% patients. All patients underwent CT or MRI of the brain, 66% patients had abnormal scans. Temporal lobe involvement was seen in 34 patients (50%) and 11 patients had purely extra temporal lesions. All patients were treated with standard Acyclovir. Seven patients died. At the time of discharge, 17 patients showed normal neurological examination, 29 were ambulatory with assistance and 15 were bedridden. CONCLUSION: Our study suggests that large number of patients with HSE have extra temporal involvement on CT or MRI. Majority of patients had complete or good recovery after completion of therapy

Clinico-laboratory findings in male lupus patients from a tertiary care hospital, Pakistan

This study was conducted to delineate the clinical pattern of a cohort of Pakistani male patients with systemic lupus erythematosus (SLE). Clinical and laboratory data were collected of 24 male patients who were diagnosed with SLE and admitted to a tertiary care hospital between 1986 and 2001. Imaging and invasive studies (including aspirations and biopsies) were also recorded. Fourteen patients (58%) had renal involvement, with WHO class 4 and 5 comprising 89% of the cases. Eight patients (33%) had neurological involvement. Out of these 8 patients, 3 presented with psychosis (12.5%) and 4 (17%) with seizures. Twenty three patients (96%) had hematological involvement, 6 (25%) had serosal and 10 (41%) had articular involvement. Cutaneous lesions were noted in 10 (41%) patients. A majority of the patients were noncompliant and were lost to follow-up; therefore, ultimate outcome could not be clearly delineated. A high index of suspicion for SLE in males may permit early diagnosis and dictate the need for more aggressive therapy

Elotuzumab plus lenalidomide and dexamethasone in relapsed/refractory multiple myeloma: extended 4-year follow-up and analysis of relative progression-free survival from the randomized ELOQUENT-2 trial

Background: The randomized phase 3 ELOQUENT-2 study (NCT01239797) evaluated the efficacy and safety of elotuzumab plus lenalidomide and dexamethasone (ELd) versus lenalidomide and dexamethasone (Ld) in relapsed/refractory multiple myeloma (RRMM), and to date, has the longest follow-up of any monoclonal antibody in patients with RRMM. Methods: In this extended 4-year follow-up of the ELOQUENT-2 trial, the coprimary endpoints of progression-free survival (PFS) and overall response rate as well as the secondary endpoint of overall survival were assessed. In the absence of head-to-head trials comparing Ld-based triplet regimens to guide treatment selection, 4 randomized controlled trials—ELOQUENT-2, ASPIRE, TOURMALINE-MM1, and POLLUX—were indirectly compared to provide insight into the relative efficacy of these regimens in RRMM. Results: Data at 4 years were consistent with 2- and 3-year follow-up data: ELd reduced the risk of disease progression/death by 29% versus Ld (hazard ratio, 0.71) while maintaining safety. The greatest PFS benefit among the assessed subgroups was observed in patients at the median time or further from diagnosis (≥3.5 years) with 1 prior line of therapy, who had a 44% reduction in the risk of progression/death, and in patients in the high-risk category, who had a 36% reduction in favor of ELd. This regimen also showed a relative PFS benefit that was maintained beyond 50 months. Conclusions: The sustained PFS benefit and long-term safety of ELd at 4 years, similar to those observed at 2 and 3 years, support ELd as a valuable therapeutic option for the long-term treatment of patients with RRMM. © 2018 The Authors. Cancer published by Wiley Periodicals, Inc. on behalf of American Cancer Societ