Additionally, four figures and legends demonstrate the patient’s lung function trajectory, the high-resolution computed tomography (CT) scan of the patient’s chest showing progression from a prior scan, an overview of the pathogenesis of SSc-ILD, along with the role of B cells in the disease and a pictorial summary of the patient case study.
Patient Case Study
The case study follows a 69-year-old man diagnosed with diffuse cutaneous SSc in 2014. The patient’s history revealed Raynaud’s symptoms in September 2013, followed by digital puffiness in January 2014. Over the next six months, he developed skin thickening on the upper limbs and chest and experienced reflux, proximal muscle weakness and weight loss. Tests around the time of his diagnosis, in August 2014, showed a modified Rodnan skin score (mRSS) of 19/51, hemoglobin 127 g/L, creatine kinase 757 IU/L, C-reactive protein (CRP) <1 mg/L, erythrocyte sedimentation rate (ESR) 2 mm/hr, N-terminal pro-B-type natriuretic peptide (NT-proBNP) 26 pmol/L (high >236) and esophageal dysmotility. HRCT confirmed ILD.
The patient started immunosuppressive therapy comprising mycophenolate mofetil, 2 g daily, for active skin disease and ILD, and prednisolone, 20 mg daily, for myositis. Later that year, he developed complete heart block, requiring a pacemaker. A 2015 echocardiogram showed a left ventricular ejection fraction of 45% and an abnormal regional wall motion with slight left ventricular apex hypokinesia. The patient’s maintenance immunosuppressive therapy comprised mycophenolate mofetil, 2 g, and prednisolone, 7.5 mg daily.
At a May 2022 follow-up appointment, the patient reported increasing shortness of breath and exercise intolerance over the prior year without substantial cough, chest pain or ankle edema. He noted recurrent urinary tract infections needing multiple antibiotic courses, but no chest infections. SSc gastrointestinal involvement, including vomiting, diarrhea and bloating, persisted despite taking a high-dose proton pump inhibitor and a histamine type-2 receptor antagonist for gastroesophageal reflux disease, prokinetics for esophageal dysmotility and rotational antibiotics for bacterial overgrowth in the small intestine. The patient maintained his weight with high-calorie supplements.
Examination revealed a stable mRSS of 9/51 and fine bilateral inspiratory crackles. Autoimmune serology showed speckled pattern anti-nuclear antibodies 1:1280, negative extractable nuclear antigen antibodies and no SSc-specific antibody. Laboratory test results showed hemoglobin 154 g/L, CRP 2mg/L and ESR 5 mm/hr. Cardiac markers included NT-proBNP 230 ng/L and troponin T 19 ng/L and an echocardiogram suggested a low likelihood of pulmonary hypertension. Lung function tests showed declines in forced vital capacity and DLCO (diffusing capacity of the lungs for carbon monoxide) by 18.2% and 11.8%, respectively, over the past 12 months.
