Chiefs’ Inquiry Corner-5/16/22

 Systemic sclerosis is a connective tissue disorder characterized by widespread fibrosis of the internal organs and skin, as well as vascular and immune system manifestations; its clinical manifestations are broad and heterogeneous, prompting some to consider it a spectrum of disorders rather than one single entity. Nonetheless, skin involvement is typically considered to be a cardinal feature of systemic sclerosis (the term ‘scleroderma’ itself means ‘hardening of the skin’), and frequently develops early in the disease course. Typical skin changes include nonpitting edema followed by skin thickening, classically in the fingertips (leading to their tapering) and the face (leading to tightening of the skin and difficulty opening the mouth). However, a rare subset of systemic sclerosis has been identified, called systemic sclerosis sine scleroderma (ssSSc), which is characterized by the total absence (or partial absence) of cutaneous manifestations of systemic sclerosis but still with internal organ involvement and serologic evidence of the disease. Of the organ systems involved, gastrointestinal and pulmonary systems are the most commonly affected (79% and 68%, respectively); cardiac involvement is rarer (9% of patients). GI manifestations include esophageal dysmotility, dysphagia, nausea, and vomiting, while pulmonary manifestations include interstitial lung disease and pulmonary hypertension. For those with cardiac manifestations, the most common ones include chronic heart failure, pericardial effusions, and conduction disturbances.  Overall, systemic sclerosis remains a difficult disease to diagnose. Clinicians should maintain a high index of suspicion for systemic sclerosis in people who appear to have internal organ involvement and serologies consistent with systemic sclerosis, even in the absence of the skin findings that are often considered to be central to its recognition and diagnosis. 

References: Systemic sclerosis sine scleroderma

 The Global Initiative for Asthma (GINA) recently released the 2022 update of the Global Strategy for Asthma Management and Prevention, based on a review of recent scientific literature by an international panel of experts. While the treatment of asthma is still guided by a ‘stepwise’ approach (with treatment escalation based on symptom severity and frequency), new data has changed the recommendation for initial asthma management. Formerly, Step 1 recommended the use of an as-needed short-acting beta2 agonist, or SABA (such as albuterol) for people with infrequent symptoms (less than 4-5 days per week). The recommendation has now changed from SABA monotherapy to SABA plus a low-dose inhaled corticosteroid (ICS) (low-dose ICS-formoterol is the preferred agent; otherwise, the person should use an inhaled corticosteroid whenever a SABA is also used). Data has shown that this therapeutic approach reduces the risk of severe exacerbations compared with regimens with SABA as monotherapy, with similar symptom control. In addition, early initiation of low-dose ICS in people with asthma leads to a greater improvement in lung function than if symptoms have been present for more than 2-4 years before initiation of ICS. It was also noted that initiation of treatment with SABA alone encouraged patients to consider the SABA their main asthma treatment, with resultant increased risk of poor adherence to daily ICS when subsequently prescribed. 

References: Global Strategy for Asthma Management and Prevention (2022 Update)

 The US Preventive Services Task Force recommends considering initiation of aspirin for prevention of cardiovascular disease in adults aged 40-59 years old  with an estimated CVD risk of 10% or greater, and who are not at increased risk for bleeding (i.e., no history of gastrointestinal ulcers, recent bleeding, or other medical conditions that confer an increased risk of bleeding, or taking other medications that increase bleeding risk). This decision should be individualized, rather than broadly initiating aspirin in all patients in the recommended age group meeting CVD risk criteria. If aspirin is to be initiated, 81mg daily is a reasonable dose. Of note, adults aged 60 or older, the recommendation is against initiation of aspirin for primary prevention. In addition, while there had been a question of whether aspirin impacted colorectal incidence or mortality, the evidence remains unclear whether aspirin use reduces the risk of either of these.

References: Aspirin Use to Prevent Cardiovascular Disease