In Times of Crisis: Delayed Diagnosis of Scleroderma Renal Crisis in Limited Systemic Sclerosis

Introduction: Scleroderma renal crisis (SRC) is a rare, life-threatening complication of systemic sclerosis (SSc) with a much lower prevalence in limited cutaneous SSc (lcSSc) (1-2%) compared to diffuse cutaneous SSc (dcSSc) (5-14%). This case report focuses on the unique aspects of SRC in patients with lcSSc and the challenges of diagnosis. Case Description: A 63-year-old female with a PMH of HTN, hypothyroidism, and Raynaud's phenomenon presented with worsening fatigue, dyspnea, and weight loss over two months. Review of symptoms was positive for nausea, vomiting, and joint pain. Physical exam was significant for crackles in bilateral lung fields and lower extremity edema. Vitals were significant for blood pressure of 170/76. Labs showed hemoglobin of 6.5 g/dL, platelet count of 83,000 mcL, and creatinine of 1.04 mg/dL. Anemia workup was suggestive of hemolytic anemia. Peripheral smear showed occasional schistocytes suggesting a microangiopathic process. Imaging showed moderate sized pericardial effusion, bilateral pleural effusions, and interstitial lung changes. Autoimmune etiology was suspected; workup was positive for antinuclear antibody (1:160) and anti-centromere antibody. Diagnosis of lcSSc (CREST syndrome) was made and patient was started on mycophenolate 500 mg BID. Acute kidney injury was noted on day 4 of admission with progressively worsening renal function. Renal biopsy confirmed acute thrombotic microangiopathy. Patient was diagnosed with SRC and started on Captopril, eventually requiring long-term hemodialysis. Discussion: In lcSSc, there is a milder and more gradual disease course which can obscure the early signs of SRC making a timely diagnosis challenging. Despite its rarity in lcSSc, SRC should always be considered when any manifestation is present because of its high mortality/morbidity if not treated promptly. Over 50% of SRC patients require renal replacement therapy, as seen in this case. Thus, here we highlight the risk of delayed SRC diagnosis and reinforce the need for early recognition to prevent irreversible outcomes such as the need for chronic hemodialysis.