A comprehensive framework for navigating patient care in systemic sclerosis: A global response to the need for improving the practice of diagnostic and preventive strategies in SSc

Abstract

Systemic sclerosis (SSc), the most lethal of rheumatologic conditions, is the cause of death in >50% of SSc cases, led by pulmonary fibrosis followed by pulmonary hypertension and then scleroderma renal crisis (SRC). Multiple other preventable and treatable SSc-related vascular, cardiac, gastrointestinal, nutritional and musculoskeletal complications can lead to disability and death. Vascular injury with subsequent inflammation transforming to irreversible fibrosis and permanent damage characterizes SSc. Organ involvement is often present early in the disease course of SSc, but requires careful history-taking and vigilance in screening to detect. Inflammation is potentially reversible provided that treatment intensity quells inflammation and other immune mechanisms. In any SSc phenotype, opportunities for early treatment are prone to be under-utilized, especially in slowly progressive phenotypes that, in contrast to severe progressive ILD, indolently accrue irreversible organ damage resulting in later-stage life-limiting complications such as pulmonary hypertension, cardiac involvement, and malnutrition. A single SSc patient visit often requires much more physician and staff time, organization, vigilance, and direct management for multiple organ systems compared to other rheumatic or pulmonary diseases. Efficiency and efficacy of comprehensive SSc care enlists trending of symptoms and bio-data. Financial sustainability of SSc care benefits from understanding insurance reimbursement and health system allocation policies for complex patients. Sharing care between recognised SSc centers and local cardiology/pulmonary/rheumatology/gastroenterology colleagues may prevent complications and poor outcomes, while providing support to local specialists. As scleroderma specialists, we offer a practical framework with tools to facilitate an optimal, comprehensive and sustainable approach to SSc care. Improved health outcomes in SSc relies upon recogntion, management and, to the extent possible, prevention of SSc and treatment-related complications.

Keywords: Disability; Interstitial lung disease; Pulmonary fibrosis; Pulmonary hypertension; Quality of life; Renal crisis; Scleroderma; Symptom burden; Systemic sclerosis.

Figure 1.

SSc involved tissue, of which the lung is one example, experiences transition from healthy tissue to fibrosis as inflammation is incited and progressively extends within resident organs. Vascular injury with tissue hypoxia is an important factor to the development of tissue fibrosis. Symptoms and disability can be transient with active inflammation with systemic treatment. Over time untreated inflammation irreparably injures effected tissue resulting in scarring and fibrosis. Fibrosis is irreversible and results in permanent organ-related disability. (Courtesy of LA Saketkoo, rights reserved)

Figure 2.

Demonstration of ‘normal’ and various SSc patterns on nail fold video capillaroscopy. (Images courtesy of Vanessa Smith; University of Ghent, Belgium.)

Figure 3.. Capillaroscopy is an essential rheumatologic service.

An abnormal capillaroscopy satisfies >20% of SSc criteria and confers 96% predictive power for development of CTD; making it an essential part of the rheumatologic exam. With any method capillaries become increasingly easier to visualize with practice over time. (Courtesy of T Frech & LA Saketkoo, rights reserved)

Figure 4.. Clinical-Serologic Classification and Internal Organ Associations

(Courtesy of RT Domsic, rights reserved) ILD = interstitial lung disease; DU = digital ulcers; SRC = scleroderma renal crisis; PH = pulmonary hypertension

Figure 5.. Depiction of the diffuse nature of gastrointestinal involvement in SSc.

Courtesy of T Frech, rights reserved.

Figure 6.

Diffusion Capacity of the Lung for Carbon Monoxide Measures the Ability of Gas Transfer (illustrations courtesy of LA Saketkoo, rights reserved.)

Figure 7.. DLCO behavior in ILD versus PH Predominance.

The closer the ratio of FVC:DLCO is to 1 the more likely abnormal changes are related to restrictive lung disease. (illustration courtesy of LA Saketkoo)

Figure 9.

The Renal Crisis Prevention Card may help patients direct emergency healthcare providers to abort a crisis and avoid adverse outcomes.

Diagram 1.

Proposed Screening and Monitoring Algorithm for Clinically Significant SSc-ILD. Courtesy of LA Saketkoo, rights reserved.