THE EARLY-ONSET SCLERODERMAL RENAL CRISIS CHALLENGE: A FATAL CASE REPORT

 

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THE EARLY-ONSET SCLERODERMAL RENAL CRISIS CHALLENGE: A FATAL CASE REPORT

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Brenda Paola
Hernández Henríquez
Brenda Paola Hernández Henríquez drabpaolahdz@gmail.com University Hospital, Universidad Autónoma de Nuevo León (UANL) Nephrology Monterrey Mexico *
Manuel Eduardo Arenas Lerma m.eduardo.arenas@gmail.com University Hospital, Universidad Autónoma de Nuevo León (UANL) Nephrology Monterrey Mexico -
Jose Fidel Veliz Escobar m.eduardo.arenas@gmail.com University Hospital, Universidad Autónoma de Nuevo León (UANL) Nephrology Monterrey Mexico -
Kenya Yamileth Rodríguez Ibarra kenya.rdzibarra@gmail.com University Hospital, Universidad Autónoma de Nuevo León (UANL) Nephrology Monterrey Mexico -
Elisa Guerrero González eliguerrero24@hotmail.com University Hospital, Universidad Autónoma de Nuevo León (UANL) Nephrology Monterrey Mexico -
Mara Olivo Gutiérrez mara_olivo84@hotmail.com University Hospital, Universidad Autónoma de Nuevo León (UANL) Nephrology Monterrey Mexico -
Giovanna Yazmin Arteaga Muller Giomuller@gmail.com University Hospital, Universidad Autónoma de Nuevo León (UANL) Nephrology Monterrey Mexico -
Mónica Sánchez Cárdenas DRA.MONICASANCHEZ87@gmail.com University Hospital, Universidad Autónoma de Nuevo León (UANL) Nephrology Monterrey Mexico -
Valeria Caballero Malacara valee.caballero94@gmail.com University Hospital, Universidad Autónoma de Nuevo León (UANL) Pathology Monterrey Mexico -
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SSc is an autoimmune that involves skin and internal organs, including kidneys. SRC is one of the most severe complications. It’s a syndrome characterized by a rapid decline in renal function, hypertensive emergency and TMA presence. SRC prevalence is low (2.6-12 cases per million inhabitants) and highly mortal if not promptly treated.

We report the case of a 49-year-old woman with 1 year history of diffuse SSc (lung, skin and joint affection), without kidney disease. She presented to the emergency department due to dyspnea of 2 weeks´ duration with fatigue, hyperthermia and anuria associated. Laboratory results demonstrated the need for urgent RRT. Table 1 provides a summary of the patient’s clinical and biochemical features.

Chest CT revealed associated interstitial lung disease, pleural and pericardial effusion. Figure 1 shows skin lesions and CT/ renal biopsy findings. Immediate antihypertensive therapy with captopril was initiated, along with renal replacement therapy, requiring up to three anihypertensive agents.

The immunosuppressive therapy was temporarily suspended until an infectious process could be ruled out. During her hospital course, she required intermittent acute hemodialysis sessions, with a rising BUN/creatinine trend, and persistent anuria, necessitating chronic renal replacement therapy. After a 1-month hospitalization, the patient died from pulmonary embolism.





Scleroderma renal crisis (SRC) is a rare complication of systemic sclerosis, more frequent in the diffuse form, which can rapidly progress to acute kidney injury, need for renal replacement therapy, and death. Timely diagnosis requires clinical correlation with systemic sclerosis, exclusion of other thrombotic microangiopathies, and, when feasible, histological confirmation via renal biopsy. Early initiation of ACE inhibitors, particularly captopril, improves short-term survival but does not guarantee renal recovery in all cases, highlighting the clinical heterogeneity and the need for predictive biomarkers and more effective therapeutic strategies.

Kewords