Hidradenitis suppurativa: Epidemiology, clinical presentation, and pathogenesis.

Hidradenitis suppurativa (HS) is an inflammatory disorder that is characterized by chronic deep-seated nodules, abscesses, fistulae, sinus tracts, and scars in the axilla, inguinal area, submammary folds, and perianal area. This disfiguring condition is accompanied by pain, embarrassment, and a significantly decreased quality of life. Although the mechanism of HS has not been entirely elucidated, lesion formation is believed to center around follicular hyperkeratosis within the pilosebaceous-apocrine unit. Recent research has provided new insight into the role of cytokines in the pathogenesis of HS, helping close some existing knowledge gaps in the development of this condition. The first article in this continuing medical education series reviews HS epidemiology, clinical presentation, and classification. We also provide an update on the most recent understanding of HS pathogenesis, including the central role of inflammatory cytokines and other contributing factors, such as genetics, hormones, and pathogenic microorganisms.

Hidradenitis suppurativa: Current and emerging treatments.

The treatment of hidradenitis suppurativa (HS) has remained challenging because of the many knowledge gaps regarding etiology. However, recent studies into the pathogenesis of HS have enabled the investigation of newer therapies. The second article in this continuing medical education series reviews the evidence for established therapies for HS, including anti-inflammatories, antibiotics, and surgery. New and emerging therapies that specifically target cytokines involved in HS pathogenesis will be covered. The potential therapeutic roles of anticytokine therapies, including both the expanded application of existing molecules as well as the specific development of novel therapies for HS are discussed. With increased attention on HS and with numerous clinical trials currently underway, we hope that the variety of treatment options for HS will be expanded.

Prognostic utility of differential tissue characterization of cardiac neoplasm and thrombus via late gadolinium enhancement cardiovascular magnetic resonance among patients with advanced systemic cancer.

BACKGROUND: Late gadolinium enhancement (LGE-) cardiovascular magnetic resonance (CMR) is well-validated for cardiac mass (CMASS) tissue characterization to differentiate neoplasm (CNEO) from thrombus (CTHR): Prognostic implications of CMASS subtypes among systemic cancer patients are unknown. METHODS: CMASS + patients and controls (CMASS -) matched for cancer diagnosis and stage underwent a standardized CMR protocol, including LGE-CMR (IR-GRE) for tissue characterization and balanced steady state free precession cine-CMR (SSFP) for cardiac structure/function. CMASS subtypes (CNEO, CTHR) were respectively defined by presence or absence of enhancement on LGE-CMR; lesions were quantified for tissue properties (contrast-to-noise ratio (CNR); signal-to-noise ratio (SNR) and size. Clinical follow-up was performed to evaluate prognosis in relation to CMASS etiology. RESULTS: The study population comprised 126 patients with systemic neoplasms referred for CMR, of whom 50% (n = 63) had CMASS + (CNEO = 32%, CTHR = 18%). Cancer etiology differed between CNEO (sarcoma = 20%, lung = 18%) and CTHR (lymphoma = 30%, GI = 26%); cardiac function (left ventricular ejection fraction: 63 ± 9 vs. 62 ± 10%; p = 0.51∣ right ventricular ejection fraction: 53 ± 9 vs. 54 ± 8%; p = 0.47) and geometric indices were similar (all p = NS). LGE-CMR tissue properties assessed by CNR (13.1 ± 13.0 vs. 1.6 ± 1.0; p < 0.001) and SNR (29.7 ± 20.4 vs. 15.0 ± 11.4, p = 0.003) were higher for CNEO, consistent with visually-assigned diagnostic categories. CTHR were more likely to localize to the right atrium (78% vs. 25%, p < 0.001); nearly all (17/18) were associated with central catheters. Lesion size (17.3 ± 23.8 vs. 2.0 ± 1.5 cm2; p < 0.001) was greater with CNEO vs. CTHR, as was systemic disease burden (cancer-involved organs: 3.6 ± 2.0 vs. 2.3 ± 2.1; p = 0.02). Mortality during a median follow-up of 2.5 years was markedly higher among patients with CNEO compared to those with CTHR (HR = 3.13 [CI 1.54-6.39], p = 0.002); prognosis was similar when patients were stratified by lesion size assessed via area (HR = 0.99 per cm2 [CI 0.98-1.01], p = 0.40) or maximal diameter (HR = 0.98 per cm [CI 0.91-1.06], p = 0.61). CTHR conferred similar mortality risk compared to cancer-matched controls without cardiac involvement (p = 0.64) whereas mortality associated with CNEO was slightly higher albeit non-significant (p = 0.12). CONCLUSIONS: Among a broad cancer cohort with cardiac masses, CNEO defined by LGE-CMR tissue characterization conferred markedly poorer prognosis than CTHR, whereas anatomic assessment via cine-CMR did not stratify mortality risk. Both CNEO and CTHR are associated with similar prognosis compared to CMASS - controls matched for cancer type and disease extent.

Echocardiographic linear fractional shortening for quantification of right ventricular systolic function-A cardiac magnetic resonance validation study.

BACKGROUND: Echocardiography (echo)-based linear fractional shortening (FS) is widely used to assess left ventricular dysfunction (LVdys ), but has not been systematically tested for right ventricular dysfunction (RVdys ). METHODS: The population comprised LVdys patients with and without RVdys (EF<50%) on cardiac MRI (CMR): Echo included standard RV indices (fractional area change [FAC], TAPSE, S', and FS in parasternal long-axis (RV outflow tract [RVOT ]) and apical four-chamber views (width [RVWD ], length [RVLG ]). RESULTS: A total of 168 patients underwent echo and CMR (3±3 days); FAC (46±9 vs 28±11), TAPSE (1.9±0.4 vs 1.5±0.3), and S' (11.4±2.3 vs 10.0±2.6, all P≤.001) were lower among RVdys patients, as were FS indices (RVOT 32±8 vs 17±10 | RVWD 40±11 vs 22±12 | RVLG 16±5 vs 9±4%; all P<.001). FS indices yielded similar magnitude of correlation with CMR RVEF (r=.73-.56) as did FAC (r=.70), which was slightly higher than TAPSE (r=.47) and S' (r=.31; all P<.001). FS indices decreased stepwise vs CMR RVEF tertiles, as did FAC (all P<.001). In multivariate analysis, FS in RVOT (regression coefficient .51 [CI 0.37-0.65]), RVWD (0.30 [0.19-0.41]), and RVLG (0.45 [0.20-0.71]; all P≤.001) was independently associated with CMR RVEF. FS indices yielded good overall diagnostic performance (AUC: RVOT 0.89 [CI 0.82-0.97] | RVWD 0.87 [0.78-0.96] | RVLG 0.80 [0.70-0.90]; all P<.001) for CMR-defined RVdy (RVEF<50%). CONCLUSIONS: RV linear FS provides RV functional indices that parallel CMR RVEF. Parasternal long-axis RVOT width, four-chamber RV width, and length are independently associated with RVEF, supporting use of multiple FS indices for RV functional assessment.

Echocardiography-quantified myocardial strain-a marker of global and regional infarct size that stratifies likelihood of left ventricular thrombus.

BACKGROUND: Myocardial strain provides a novel means of quantifying subtle alterations in contractile function; incremental utility post-MI is unknown. OBJECTIVES: To test longitudinal-quantified by postprocessing routine echo-for assessment of MI size measured by cardiac magnetic resonance (CMR) and conventional methods, and assess regional and global strain (GLS) as markers of LV thrombus. METHODS: The population comprised of patients with anterior ST-segment MI who underwent echo and CMR prospectively. Preexisting echoes were retrieved, re-analyzed for strain, and compared to conventional MI markers as well as CMR-evidenced MI, function, and thrombus. RESULTS: Seventy-four patients underwent echo and CMR 4 ± 1 weeks post-MI; 72% had abnormal GLS. CMR-quantified MI size was 2.5-fold larger and EF lower among patients with abnormal GLS, paralleling 2.6-3.1 fold differences in Q-wave size and CPK (all P ≤ .002). GLS correlated with CMR-quantified MI (r = .66), CPK (r = .52) and Q-wave area (r = .44; all P ≤ .001): Regional strain was lower in the base, mid, and apical LV among patients with CMR-defined transmural MI in each territory (P < .05) and correlated with cine-CMR regional EF (r = .53-.71; P < .001) and echo wall motion (r = .45-.71; P < .001). GLS and apical strain were ~2-fold lower among patients with LV thrombus (P ≤ .002): Apical strain yielded higher diagnostic performance for thrombus (AUC: 0.83 [0.72-0.93], P = .001) than wall motion (0.73 [0.58-0.88], P = .02), as did global strain (0.78 [0.65-0.90], P = .005) compared to LVEF (0.58 [0.45-0.72], P = .41). CONCLUSIONS: Echo-quantified longitudinal strain provides a marker of MI size and improves stratification for post-MI LV thrombus beyond conventional indices.

Secondary Multiple Evanescent White Dot Syndrome Following Blunt Trauma.

PURPOSE: To report a case of secondary unilateral MEWDS following blunt trauma to the eye. METHODS: Observational case report of one patient. RESULTS: A 25-year-old male initially presented after being hit in the left eye with a football, with visual acuity of 20/50, traumatic iritis, commotio retinae, vitreous hemorrhage, and a large pigment epithelial detachment in the superior macula. He was lost to follow-up for two years before presenting with a sudden decrease in vision. On exam, best corrected VA (BCVA) was counting fingers and clinical exam demonstrated fibrosis in the superior macula, small white lesions around the optic disc and throughout the posterior pole, and ellipsoid zone disruption on OCT. Given the clinical appearance, a diagnosis of Multiple Evanescent White Dot Syndrome was made and the decision was made to observe. Two months later, without treatment, BCVA improved to 20/20, and there was resolution of the white lesions in the posterior pole as well as improved continuity of the ellipsoid zone on OCT. DISCUSSION: We describe a case of Multiple Evanescent White Dot Syndrome two years after blunt trauma to the eye; a far longer latency than previously reported cases of MEWDS secondary to blunt trauma.

Expanded Spectrum Torpedo Retinopathy: A Case Series.

Torpedo lesions in the retina are rare, and their clinical significance has not been well established. This case series highlights patients with atypical torpedo lesions with varying orientations and pigmentation patterns. We describe the first documented case of an inferiorly oriented lesion, to our knowledge, and add to the few previous descriptions of double-torpedo lesions.

Repair of a Tractional Retinal Detachment in the Setting of an Idiopathic Vasoproliferative Tumor.

Vasoproliferative tumors (VPT) are benign retinal lesions that may cause epiretinal membrane proliferation and tractional retinal detachments (TRD). We describe a case of a 71-year-old woman who presented with a macula involving TRD in the setting of a VPT. Given the limited number of publications on the management of these cases, we aim to articulate some principles we believe may be helpful in planning a surgical approach that maximizes postoperative anatomic and functional outcomes. We hope that our video provides useful guidance in preparing the vitreoretinal surgeon for managing this uncommon entity. [Ophthalmic Surg Lasers Imaging Retina 2023;54:485-488.].

Prelimbic cortex drives discrimination of non-aversion via amygdala somatostatin interneurons.

The amygdala and prelimbic cortex (PL) communicate during fear discrimination retrieval, but how they coordinate discrimination of a non-threatening stimulus is unknown. Here, we show that somatostatin (SOM) interneurons in the basolateral amygdala (BLA) become active specifically during learned non-threatening cues and desynchronize cell firing by blocking phase reset of theta oscillations during the safe cue. Furthermore, we show that SOM activation and desynchronization of the BLA is PL-dependent and promotes discrimination of non-threat. Thus, fear discrimination engages PL-dependent coordination of BLA SOM responses to non-threatening stimuli.

Ischemic Mitral Regurgitation: Abnormal Strain Overestimates Nonviable Myocardium.

BACKGROUND: Therapy for moderate ischemic mitral regurgitation remains unclear. Determination of myocardial viability, a necessary prerequisite for an improvement in regional contractility, is a likely key factor in determining response to revascularization alone. Myocardial strain has been proposed as a viability measure but has not been compared with late gadolinium enhancement (LGE) cardiac magnetic resonance imaging. We hypothesized that abnormal strain overestimates nonviable left ventricular (LV) segments measured with LGE and that ischemia and mechanical tethering by adjacent transmural myocardial infarction (TMI) also decreases strain in viable segments. METHODS: Sixteen patients with mild or greater ischemic mitral regurgitation and 7 healthy volunteers underwent cardiac magnetic resonance imaging with noninvasive tags (complementary spatial modulation of magnetization [CSPAMM]), LGE, and stress perfusion. CSPAMM images were post-processed with harmonic phase and circumferential and longitudinal strains were calculated. Viability was defined as the absence of TMI on LGE (hyperenhancement >50% of wall thickness). The borderzone was defined as any segment bordering TMI. Abnormal strain thresholds (±1 to 2.5 SDs from normal mean) were compared with TMI, ischemia, and borderzone. RESULTS: 7.4% of LV segments had TMI on LGE, and more than 14.5% of LV segments were nonviable by strain thresholds (p < 0.005). In viable segments, ischemia impaired longitudinal strain (least perfused one-third of LV segments: -0.18 ± 0.08 versus most perfused: -0.22 ± 0.1, p = 0.01) and circumferential strain (-0.12 ± 0.1 versus -0.16 ± 0.08, p < 0.05). In addition, infarct proximity impaired longitudinal strain (-0.16 ± 0.11 borderzone versus -0.18 ± 0.09 remote, p = 0.05). CONCLUSIONS: Impaired LV strain overestimates nonviable myocardium compared with TMI on LGE. Ischemia and infarct proximity also decrease strain in viable segments.

Multiplanar strain quantification for assessment of right ventricular dysfunction and non-ischemic fibrosis among patients with ischemic mitral regurgitation.

BACKGROUND: Ischemic mitral regurgitation (iMR) predisposes to right ventricular (RV) pressure and volume overload, providing a nidus for RV dysfunction (RVDYS) and non-ischemic fibrosis (NIF). Echocardiography (echo) is widely used to assess iMR, but performance of different indices as markers of RVDYS and NIF is unknown. METHODS: iMR patients prospectively underwent echo and cardiac magnetic resonance (CMR) within 72 hours. Echo quantified iMR, assessed conventional RV indices (TAPSE, RV-S', fractional area change [FAC]), and strain via speckle tracking in apical 4-chamber (global longitudinal strain [RV-GLS]) and parasternal long axis orientation (transverse strain). CMR volumetrically quantified RVEF, and assessed ischemic pattern myocardial infarction (MI) and septal NIF. RESULTS: 73 iMR patients were studied; 36% had RVDYS (EF<50%) on CMR among whom LVEF was lower, PA systolic pressure higher, and MI size larger (all p<0.05). CMR RVEF was paralleled by echo results; correlations were highest for RV-GLS (r = 0.73) and lowest for RV-S' (r = 0.43; all p<0.001). RVDYS patients more often had CMR-evidenced NIF (54% vs. 7%; p<0.001). Whereas all RV indices were lower among NIF-affected patients (all p≤0.006), percent change was largest for transverse strain (48.3%). CMR RVEF was independently associated with RV-GLS (partial r = 0.57, p<0.001) and transverse strain (r = 0.38, p = 0.002) (R = 0.78, p<0.001). Overall diagnostic performance of RV-GLS and transverse strain were similar (AUC = 0.93[0.87-0.99]|0.91[0.84-0.99], both p<0.001), and yielded near equivalent sensitivity and specificity (85%|83% and 80%|79% respectively). CONCLUSION: Compared to conventional echo indices, RV strain parameters yield stronger correlation with CMR-defined RVEF and potentially constitute better markers of CMR-evidenced NIF in iMR.

Right Ventricular Dysfunction Impairs Effort Tolerance Independent of Left Ventricular Function Among Patients Undergoing Exercise Stress Myocardial Perfusion Imaging.

BACKGROUND: Right ventricular (RV) and left ventricular (LV) function are closely linked due to a variety of factors, including common coronary blood supply. Altered LV perfusion holds the potential to affect the RV, but links between LV ischemia and RV performance, and independent impact of RV dysfunction on effort tolerance, are unknown. METHODS AND RESULTS: The population comprised 2051 patients who underwent exercise stress myocardial perfusion imaging and echo (5.5±7.9 days), among whom 6% had echo-evidenced RV dysfunction. Global summed stress scores were ≈3-fold higher among patients with RV dysfunction, attributable to increments in inducible and fixed LV perfusion defects (all P≤0.001). Regional inferior and lateral wall ischemia was greater among patients with RV dysfunction (both P<0.01), without difference in corresponding anterior defects (P=0.13). In multivariable analysis, inducible inferior and lateral wall perfusion defects increased the likelihood of RV dysfunction (both P<0.05) independent of LV function, fixed perfusion defects, and pulmonary artery pressure. Patients with RV dysfunction demonstrated lesser effort tolerance whether measured by exercise duration (6.7±2.8 versus 7.9±2.9 minutes; P<0.001) or peak treadmill stage (2.6±0.9 versus 3.1±1.0; P<0.001), paralleling results among patients with LV dysfunction (7.0±2.9 versus 8.0±2.9; P<0.001|2.7±1.0 versus 3.1±1.0; P<0.001 respectively). Exercise time decreased stepwise in relation to both RV and LV dysfunction (P<0.001) and was associated with each parameter independent of age or medication regimen. CONCLUSIONS: Among patients with known or suspected coronary artery disease, regional LV ischemia involving the inferior and lateral walls confers increased likelihood of RV dysfunction. RV dysfunction impairs exercise tolerance independent of LV dysfunction.