Ultraselective angioembolisation of splenic artery for atraumatic spleen rupture in a patient of systemic lupus erythematosus.

A systemic lupus erythematosus (SLE) patient in her mid-30s presented with spontaneous splenic haematoma and rupture. She rapidly deteriorated despite packed red cells and fresh frozen plasma transfusions. She underwent emergent ultraselective angioembolisation of the splenic artery and got stabilised. Spontaneous or atraumatic splenic rupture is rare in SLE and splenic artery embolisation may be life-saving.

Anti-SRP-positive necrotising myopathy concurrent with breast malignancy.

Anti-signal recognition particle (anti-SRP)-positive necrotising myopathy causes severe progressive proximal weakness with a propensity to involve pharyngeal, laryngeal and respiratory muscles. It is one of the aggressive inflammatory myopathies. First-line treatment is with high-dose steroids followed by other immunosuppressants, but this conventional therapy is often ineffective. Second-line treatment involves use of either rituximab or intravenous immunonoglobulin (IVIG). Anti-SRP-positive necrotising myopathy is frequently treated as refractory myositis due to its poor responsiveness to steroid monotherapy and conventional immunosuppressive therapies. Therefore, anti-SRP-positive necrotising myopathy differs from immune-mediated myopathy. Although anti-SRP autoantibody is found in only 4-6% of patients with idiopathic inflammatory myopathy, the actual proportion of patients with refractory anti-SRP-positive necrotising myopathy is unknown. We describe a patient with multiple comorbidities who had subacute-onset anti-SRP-positive immune-mediated necrotising myopathy (IMNM). After failing steroids, methotrexate and IVIG therapy, she made a considerable recovery with rituximab. She was later diagnosed to have breast malignancy. Malignancy-associated anti-SRP-positive IMNM is rarely reported.

Takayasu Arteritis in the Pre-Pulseless Phase Presenting as Pyrexia of Unknown Origin.

Pyrexia of unknown origin (PUO) or fever of unknown origin (FUO) is clinically challenging for a treating physician; it is also a conundrum for the patient until a definitive diagnosis is made. Despite extensive investigations, many cases of PUO may remain undiagnosed for a long time. In a resource-limited country like India, due to the limited availability of various diagnostic tests, a great many fever cases are classified as PUO. Here, we present a case report of Takayasu arteritis in its pre-pulseless phase, presented as PUO. Takayasu arteritis presenting as PUO in the absence of a pulse deficit is uncommon and rarely reported. The patient's fever responded to steroids with methotrexate. The patient didn't develop any vascular complications during the follow-up.

Disseminated tuberculosis in rare association with hemophagocytic lymphocytosis - A case report from central India.

Hemophagocytic Lymphohistiocytosis (HLH) is an uncommon, diverse and rare genetic hyper-inflammatory syndrome. HLH associated with tuberculosis (TB-HLH) has been described as a clinical and diagnostic quandary. The co-existence leads to significantly higher morbidity and mortality. Our case highlights the presence of disseminated tuberculosis and worsening of the case due to underlying hemophagocytic syndrome leading to rapid deterioration of patient prognosis. Prompt diagnosis and treatment remains help to improve patient management.

Aetiologies of Acute Complications in Autoimmune Rheumatologic Diseases: A Hospital-Based Cross-Sectional Study.

BACKGROUND: Autoimmune rheumatic diseases (ARD) present unique challenges in clinical practice. Many of them present in medical emergencies in an unstable state and need immediate evaluation for further plans of action. The clinical conundrum is to distinguish between sepsis, disease flare, or Addisonian crisis (AC) (secondary to steroid withdrawal). This may be further complicated by overlapping clinical features like shock/fever and the coexistence of a combination of the above pathophysiologic mechanisms (e.g. AC with sepsis or AC with disease flare). The known biomarkers may not perform optimally to distinguish them and additional supportive investigations like imaging, cultures, autoimmune serological markers, etc. are needed. Ultimately the boundaries between "the art of medicine" and "the science of medicine" may get blurred, as the established literature evidence falls short and the expert opinion is needed in a time-sensitive manner. In this pragmatic study, researchers have attempted to explore the presentation of rheumatologic emergencies on the above three differentials (sepsis, disease flare, and AC). MATERIALS AND METHODS:  In this hospital-based cross-sectional study, adult patients (age >18 years) with ARD who had unplanned hospital admission due to acute worsening were enrolled. This study was conducted over one year, after getting the Institutional Human Ethics Committee's approval. All relevant hematological, immunological, and hormonal parameters (specifically morning cortisol) were collected and analyzed. The aim was to find the individual and combined prevalence of sepsis, disease flare, or AC in this study group. RESULTS: Forty-one patients were analyzed, with females in the majority (95%) and the dominant age group being 26-49 years (56.1%). A majority had a diagnosis of rheumatoid arthritis (RA) (56.1%) or systemic lupus erythematosus (SLE) (31.7%); the rest were other connective tissue diseases (12.2%). High-risk Quick Sequential Organ Failure Assessment score (qSOFA) score 2-3 was present in 29.3% while the rest had low-risk scores (qSOFA score 0-1). Thirty-two percent had severe disease activity, 46% had mild to moderate disease activity, and 22% of patients had no disease activity. While 78% of patients had low procalcitonin (PCT) values <0.5 microgm/L (low risk of sepsis), 15% had <20 microgm/L, and 7% percentage of patients had serum levels >20 microgm/L (high risk of sepsis). A total of 73.2% of patients had no evidence of infection while 26.8% had either microbiological/radiological evidence of infection. Only 7% of all patients had the presence of an AC. qSOFA scores didn't statistically correlate with a diagnosis of infection or AC but positively correlated with PCT and C-reactive protein (CRP) values. Serum PCT didn't correlate with the presence of infection with statistically significance (p-value 0.217). CONCLUSION: Infections and sepsis are the most important considerations in the emergency presentations of ARDs. Disease flare and AC are also important differentials. Current inflammatory biomarkers like serum CRP and PCT may be less valuable for discriminating between infectious and non-infectious sepsis, especially in chronic inflammatory diseases like ARDs. qSOFA scores may have a prognostic role with less discriminant value. Management of ARD emergencies needs better biomarkers and more research is warranted.

The Significant Morbidity and Mortality Indicators in Patients of Cirrhosis.

BACKGROUND: Cirrhosis progression varies greatly from patient to patient due to a variety of factors, including hepatic reserve, cirrhosis etiology, and the presence of hepatocellular cancer. As a result, determining a prognosis in a patient with cirrhosis remains a difficult task. For nearly three decades, the Child-Pugh score (CPS) has been the gold standard for determining the prognosis of cirrhosis. In the last two decades, many prognostic models and scores like a model for end-stage liver disease (MELD), chronic liver failure-sequential organ failure assessment (CLIF-SOFA) score, peripheral blood lymphocyte to monocyte ratio (LMR) have been presented to predict prognosis in patients with cirrhosis and to choose the best therapy option. The aim of our study is to determine which score is more effective in predicting three-month mortality and whether these scores are equally effective in predicting short-term outcomes. MATERIALS & METHODS: In this hospital-based longitudinal study, we analyzed 140 patients with cirrhosis of liver visiting All India Institute of Medical Sciences Bhopal between July 2019 and July 2020. All the 140 patients were followed up for three months to establish short-term outcomes. The blood investigations were done at the time of presentation from all the patients and after three months in the survivors. Various scores were calculated. RESULTS: The majority of patients (47%) were in Child-Pugh class C. Mean MELD score was 13.54, LMR score was 1.96 and CLIF-SOFA score was 5. The total bilirubin, serum creatinine, international normalized ratio (INR), total leukocyte count, absolute monocyte count, CPS, MELD, CLIF-SOFA were significantly higher in a non-surviving group as compared to the surviving group, whereas the albumin and LMR significantly decreased in the non-surviving group. On performing multivariate regression, LMR and CLIF-SOFA were significant independent risk factors of mortality after adjusting for confounding factors. All the parameters had significant discriminatory power to predict mortality. Discriminatory power of CLIF-SOFA (AUC 0.808; 95% CI: 0.733 to 0.870) was excellent and discriminatory power of CPS (AUC 0.792; 95% CI: 0.716 to 0.856), MELD score (AUC 0.765; 95% CI: 0.685 to 0.832) and LMR (AUC 0.75; 95% CI: 0.669 to 0.819) was acceptable. Among all the parameters, CLIF-SOFA was the best predictor of mortality at a cut-off point of >5 with 80.80% chances of correctly predicting mortality. CONCLUSION: The significant morbidity and mortality indicators are high total bilirubin, high creatinine, high INR, high TLC, low platelet count, and low albumin. Among the various scores, CLIF-SOFA is a better predictor of mortality and morbidity. Low LMR and high CLIF-SOFA are significant independent risk factors of mortality at three months.

Exploring the Subclinical Atherosclerotic Load in Patients With Rheumatoid Arthritis: A Cross-Sectional Study.

BACKGROUND: Atherosclerosis is the major etiopathogenic factor that decides cardiovascular mortality and morbidity. While inflammation is the putative mechanism for atherosclerosis in various experimental studies, chronic inflammatory state (e.g. in rheumatoid arthritis [RA]) is often neglected as a contributing factor for the development of atherosclerosis. RA patients have two to four times more risk of fatal or non-fatal cardiovascular events, which is not explained by traditional risk factors alone. For example, low-density lipoprotein (LDL) cholesterol levels may not convey the true atherosclerotic risk in RA patients - "the lipid paradox". Thus, for better risk stratification of future cardiovascular events in RA, the traditional parameters like diabetes, hypertension, and dyslipidemia may not suffice. Newer parameters like carotid intimal-medial thickness (CIMT), coronary calcification scores, and C-reactive protein (CRP) may be needed. This study determined subclinical atherosclerotic load in groups of RA and non-RA patients with comparable Framingham risk scores using CIMT values. MATERIALS AND METHODS:  In this hospital-based cross-sectional study, the RA study group had 64 patients with RA (disease duration > 1 year) and 64 controls were patients with at least one traditional risk factor of cardiovascular disease (e.g., hypertension, cigarette smoking, dyslipidemia, and diabetes mellitus). They were all analyzed for CIMT. The aim was to compare if there was a difference in CIMT scores between groups of RA and non-RA patients, with comparable Framingham score cardiovascular risk categories. RESULTS: CIMT was significantly higher in the study population compared to controls, indicating increased subclinical atherosclerotic load in the former. Mean CIMT was higher in all age groups in RA patients when compared to the control population (statistically significant in age groups 40-49 years 0.66 ± 0.07 mm vs 0.64 ± 0.06 mm, P < 0.026 and 50-59 years 0.8 ± 0.05 mm vs 0.76 ± 0.05 mm, P < 0.047). CIMT was significantly higher in the intermediate-risk groups (based on the Framingham risk score) in the RA study population when compared with the same risk categories of the control population. Atherogenic indices such as LDL/high-density lipoprotein (HDL) ratio, atherogenic index, and CIMT were significantly higher in the RA patients with more than five years of disease duration than those with a duration of fewer than five years. CONCLUSION: Subclinical atherosclerotic load is higher in RA versus controls. The mean CIMT was higher in all age groups in RA compared to the controls. CIMT was significantly higher in the intermediate-risk subgroup (by Framingham risk score) when compared between RA and controls. RA subgroup comparisons based on seropositivity/seronegativity, high/normal CRP, and disease activity (low, intermediate, and high) for CIMT were not found to have statistically significant differences. RA group had lower HDL cholesterol and comparable LDL cholesterol values compared to controls.

Impact of an Anti-Microbial Stewardship Program on Targeted Antimicrobial Therapy in a Tertiary Care Health Care Institute in Central India.

Introduction Antimicrobial resistance (AMR) has become a global pandemic. In order to identify this menace, World Health Organisation (WHO) has developed the Global Action Plan on AMR (GAP AMR). Antimicrobial stewardship programs (AMSP) have been identified as a decisive tool for combating AMR. One of the most efficient measures of these programs has been the implementation of point prevalence surveys (PPS) of antibiotic usage and subsequent audit feedback. The present study was undertaken to identify the impact of AMSP on curtailing of empirical usage of antibiotics and the augmentation of targeted therapy.  Methods It is an observational, cross-sectional study comprising 1396 patients. The microbiology culture details and anti-microbial-sensitivity results were recorded. Antibiotic prescriptions were recorded in each patient during their hospital stay. Result Out of 1396 patients treated over four quarters (Q1-Q4), 711 (50.9%) patients were on antibiotics, and among them, only 415 patients were subjected to any microbiological cultures with an overall bacterial culture rate (BCR) of 58.3%, and 296 patients (41.6%) were treated with antibiotics empirically without sending any samples for bacterial culture. There was a statistically significant rise in BCR from 47.3% in the first quarter to 77.6% in the fourth quarter. Sending specimens for blood culture increased significantly from 29.2% in Q1 to 37.6% in Q4. After receiving culture reports, 72.3% of cases continued with the same antibiotic, the antibiotic was changed in 19.9% of cases, and the antibiotic was stopped in 7.8% of cases. Conclusion There was a strong positive impact of AMSP in curtailment of empirical usage of antibiotics and augmenting targeted therapy as evidenced by the significant rise in BCR over Q1-Q4 PPS as well as a significant rise in ordering for blood culture over the same time period.

Eight-and-a-half syndrome: a rare presentation.

Eight-and-a-half syndrome is a rare entity characterised by conjugate horizontal gaze palsy, ipsilateral internuclear ophthalmoplegia and ipsilateral lower motor neuron type facial palsy. It is due to a lesion affecting median longitudinal fasciculus, paramedian pontine reticular formation and facial nerve fascicle on the same side at the level of pons. The diagnosis is easily missed as it needs detailed ocular movement examination. It is mainly caused due to infarction or demyelinating conditions. We are reporting an interesting case of a 54-year-old man with right-side eight-and-a-half syndrome due to acute ischaemic stroke and ST-elevation myocardial infarction of the inferior wall.

Nephrocalcinosis-A gateway to the Diagnosis.

Nephrocalcinosis (NC) is the augmented calcium content within the renal parenchyma. Its pathogenesis mainly involves hypercalciuria. The presence of medullary NC provides a window to the clinician for the diagnosis of many important diseases. In this case series, we highlight three diseases that could be diagnosed with a high index of suspicion and detailed evaluation after their presentation as medullary NC.

Screening of Patients with Chronic Medical Disorders in the Outpatient Department for Depression Using Handheld Computers as Interface and Patient Health Questionnaire-9 as a Tool.

BACKGROUND AND OBJECTIVES: Depression, especially in concurrence with chronic medical disorders, is highly prevalent worldwide. An average between 9.3% and 23% of patients with one or more chronic disease have co-morbid depression. This comorbid depression has the worst health scores of all the disease states. Despite this, patients with chronic medical disorders are not commonly screened for depression. Lack of objective screening by health-care providers as well as lack of infrastructure (time/space/personnel) probably contributes to gross underdiagnosis of depression. This issue can be addressed using short objective depression screening score (Patient Health Questionnaire-9 [PHQ-9]) (validated in native languages, e.g., Hindi) and paperless self-administered interface on handheld computer (tablet), which is the objective of the study. SUBJECTS AND METHODS: One hundred consecutive patients with chronic medical disorders visiting our medicine outpatient department were screened for depression using tablets with PHQ-9 Hindi on a self-administered interface. RESULTS: The overall prevalence of depression was found to be 25% (95% confidence interval 16.6-34.8). Nearly half of the patients with depression had moderate depression (PHQ-9 score 10-14) while rest had moderately severe or very severe depression (PHQ-9 score >14). Association of depression was not found to be statistically significant with age, duration of disease, gender, the type of disease, or the number of disease. Majority of patients rated ease of the use of tablet interface (on a visual analog scale) as very easy (approx 95%). All the patients were able to complete the tablet screener without assistance, answering all of the questions. The median time of completion with interquartile range was 4 (3-5) min. Majority of the patients (63%) completed the questionnaire within 5 min while rest completed it in 5-10 min. CONCLUSIONS: It is feasible to use tablets with PHQ-9 questionnaire in native language for screening depression in chronic medical disorders. With high prevalence of comorbid depression, any comprehensive care of patients with chronic medical disorders will not be possible, unless such patients are screened and treated for depression. A self-administered screening questionnaire for depression on handheld tablets can prove to be a handy tool to achieve above aim.

Subcutaneous panniculitis-like T-cell lymphoma with macrophage activation syndrome treated by cyclosporine and prednisolone.

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL; α/ß T-cell subtype) is a distinct variantof cutaneous T-cell lymphomas, which presents as inflammatory subcutaneous nodules. A 17-year-old male presented with recurrent fever with concomitant facial swelling, pedal edema, hepatosplenomegaly, and mildly tender subcutaneous plaques in generalized distribution along with patches of scarring alopecia on scalp. There were features of macrophage activation syndrome in the form of hemophagocytosis in the bone marrow, pancytopenia, high serum lactate dehydrogenase levels, low fibrinogen clotting activity, prolonged activated prothrombine time (aPTT), increased serum ferritin, hypoalbuminemia, and hypertriglyceridemia. Histopathology showed lobular panniculitis-like infiltration by atypical lymphocytes rimming the adipocytes. Immunohistochemistry revealed positive CD3 and CD8 markers, whereas CD4, CD56, and CD20 were negative, consistent with the diagnosis of α/ß type of SPTCL. Treatment with oral prednisolone (1mg/kg/day) and cyclosporine (2mg/kg/day; 100 mg) led to rapid subsidence of fever, plaques, and abnormal hematological parameters within a few weeks.

Where art thou pituitary?

A middle-aged woman presented with premature menopause and recurrent episodes of vomiting with hyponatraemia. Since primary causes of hormone deficiencies were the only studies initially evaluated, there was a delayed diagnosis. Pituitary tropic hormones (serum thyroid stimulating hormone/follicle stimulating hormone/luteinising hormone), inappropriately low for low-level of target hormones (free thyroxin/oestradiol), led to detailed evaluation of trophic hormone deficiencies by pituitary stimulation. The pituitary stimulation by insulin challenge test confirmed secondary (pituitary) hypofunction. Pituitary imaging revealed an uncommon cause of hypopituitarism, which is discussed in this case report.