Abstract
We present the case of a 63-year-old female diagnosed with atypical SSc in the setting of acute SRC. She was undergoing work-up for progressive dyspnoea in the outpatient setting when she was found to have newly diagnosed restrictive lung pathology and worsening renal function, thus prompting acute hospital admission. Given multisystem involvement of the pulmonary and renal systems, the differential diagnosis included autoimmune and connective tissue disorders. Although serologies were non-specific, renal biopsy confirmed scleroderma renal disease, and she was started on treatment with captopril. This case highlights the importance of clinical judgment and timely diagnosis, even when laboratory data might indicate otherwise. LEARNING POINTS: Scleroderma renal crisis (SRC) remains an important cause of morbidity and mortality in systemic sclerosis (SSc), and clinicians should have a high index of suspicion to diagnose it.The absence of specific serologic markers makes SSc diagnosis challenging and necessitates reliance on clinical findings and additional diagnostic tools such as imaging studies and tissue sampling.