Sunday, April 30, 2017

Giant cell myocarditis review

A few points of interest form the paper:

Historically, GCM and cardiac sarcoidosis were often conflated, as both presented in similarly aged patients and could result in a myocarditis characterized by giant cells and granulomas. However, it has since been established that the two are distinct clinicopathologic entities with significant differences in presentation, histologic features, and prognosis…

The disease course is rapid, with a median time of 3 weeks from symptom onset to hospital presentation.6 While GCM appears to have no sex predilection, approximately 20% of cases occur in patients with an autoimmune disorder such as inflammatory bowel disease, celiac disease, thyroiditis, or rheumatoid arthritis—among others.4,10,11 Some cases of GCM are associated with tumors, most often thymoma and lymphoma…

Without appropriate immunosuppressive therapy, the median survival from GCM symptom onset to death or transplant is only 3 months.6 With appropriate immunosuppressive therapy, the 5-year survival rate free of transplant ranges from 52% to 72%.11,15 While the optimal immunosuppressive regimen remains to be defined, a combined double- or triple-drug cyclosporine-based therapy reportedly leads to a partial clinical remission in two-thirds of patients….

Saturday, April 29, 2017

The Coombs test: some things you need to know

A nice free full text review in the Archives of Pathology and Laboratory Medicine.

Friday, April 28, 2017

Review of carcinoid heart disease

The full text of this review is available only by subscription, but the audio summary is open access.

Here are a few key points:

Carcinoid tumors (CT) tend to grow slowly and remain asymptomatic for long periods. Carcinoid syndrome (CS), the clinical state resulting from release of mediators by the tumor, generally does not occur until it metastasizes to the liver, though there are exceptions to this rule. CS consists of vasomotor disturbances, flushing, bronchospasm and diarrhea and is primarily mediated by serotonin although other mediators including kinins, histamine and prostaglandins are involved.

Carcinoid heart disease (CHD) is a valvulopathy (most frequently the tricuspid, followed in order of frequency by the pulmonic and then the left sided valves) caused by an inflammatory and fibroproliferative response of valvular endocardium to the circulating mediators, most notably serotonin. Regurgitation is the primary lesion affecting the valves although stenosis can occur to a lesser degree.

It is estimated that 50% of patients with CS go on to develop CHD though that number may be decreasing with improvements in recognition and treatment of CS. Once CHD develops it tends to progress rapidly and worsens the prognosis of patients with CS.

Transcatheter embolization and surgical debulking of liver metastases are indicated in some patients but become less viable options if hepatic congestion has developed due to the risk of acute liver failure and bleeding.

Valve replacement may improve the outlook for selected patients.

Chronic calcium blocker use and reduced mortality in sepsis

From a recent paper:


Objectives: Experimental studies suggest that calcium channel blockers can improve sepsis outcome. The aim of this study was to determine the association between prior use of calcium channel blockers and the outcome of patients admitted to the ICU with sepsis.

Design: A prospective observational study.

Setting: The ICUs of two tertiary care hospitals in the Netherlands.

Patients: In total, 1,060 consecutive patients admitted with sepsis were analyzed, 18.6% of whom used calcium channel blockers.

Interventions: None.

Measurements and Main Results: Considering large baseline differences between calcium channel blocker users and nonusers, a propensity score matched cohort was constructed to account for differential likelihoods of receiving calcium channel blockers. Fifteen plasma biomarkers providing insight in key host responses implicated in sepsis pathogenesis were measured during the first 4 days after admission. Severity of illness over the first 24 hours, sites of infection and causative pathogens were similar in both groups. Prior use of calcium channel blockers was associated with improved 30-day survival in the propensity-matched cohort (20.2% vs 32.9% in non-calcium channel blockers users; p = 0.009) and in multivariate analysis (odds ratio, 0.48; 95% CI, 0.31–0.74; p = 0.0007). Prior calcium channel blocker use was not associated with changes in the plasma levels of host biomarkers indicative of activation of the cytokine network, the vascular endothelium and the coagulation system, with the exception of antithrombin levels, which were less decreased in calcium channel blocker users.

In the discussion section the authors mention a possible mechanism:

Here, we show a significantly reduced mortality in ICU patients who were on chronic CCB treatment before development of sepsis. The association between prior CCB use and reduced sepsis mortality was consistent in sensitivity and subgroup analyses. The mechanism by which chronic CCB use may influence sepsis outcome was not revealed by sequential measurements of host response biomarkers reflecting activation of the cytokine network, the vascular endothelium or the coagulation system, and rather may involve partial prevention of cellular toxicity related to sustained elevations in intracellular Ca2+ levels.

Update on the clinical and pathologic aspects of ANCA diseases