A Case of Atypical Scleroderma Renal Crisis

Area of Science:

• Rheumatology
• Nephrology
• Pulmonology

Background:

• Scleroderma renal crisis (SRC) is a significant complication of systemic sclerosis (SSc).
• Early diagnosis is vital for managing morbidity and mortality associated with SRC.
• Atypical presentations can challenge timely diagnosis.

Purpose of the Study:

• To highlight a case of atypical systemic sclerosis (SSc) presenting with acute scleroderma renal crisis (SRC).
• To emphasize the importance of clinical acumen in diagnosing SRC when serological markers are non-specific.
• To underscore the utility of diagnostic tools beyond laboratory tests.

Main Methods:

• Case report of a 63-year-old female with progressive dyspnea.
• Evaluation of restrictive lung pathology and worsening renal function.
• Renal biopsy for diagnosis confirmation.
• Treatment initiated with captopril.

Main Results:

• Diagnosis of atypical systemic sclerosis (SSc) in the context of acute scleroderma renal crisis (SRC).
• Renal biopsy confirmed scleroderma renal disease.
• Patient received captopril treatment.

Conclusions:

• Scleroderma renal crisis (SRC) is a critical concern in systemic sclerosis (SSc) management.
• Clinicians must maintain a high index of suspicion for SRC.
• Diagnosis often relies on clinical findings and investigations like imaging and biopsies due to non-specific serologies.