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Abstract: TH-PO637

Systemic Sclerosis Sine Syndrome: Another Cardio Renal Collusion!

Session Information

Category: Nephrology Education

  • 1302 Fellows and Residents Case Reports

Authors

  • Chen, Panpan, Albany Medical College, Albany, New York, United States
  • Bilal, Anum, Albany Medical College, Albany, New York, United States
  • Salman, Loay H., Albany Medical College, Albany, New York, United States
  • Monrroy, Mauricio, Albany Medical College, Albany, New York, United States
  • Chaudhry, Rafia I., Albany Medical College, Albany, New York, United States
Background

SSc is a diffuse connective tissue disorder with cutaneous and visceral involvement, presenting with characteristic skin sclerosis. Systemic Sclerosis sine scleroderma (ssSSC) is a rare (<10%) subset of SSc with visceral and immunologic features of SSc without skin involvement. Additionally, hypocomplementemia can be found in 20% cases of SSc, and may represent an “Overlap Syndrome”, i.e. second autoimmune condition. Scleroderma renal crisis occurs in 5-20% of SSc, and diagnosis can be missed without the characteristic sclerotic skin findings of SSc.

Methods

A 26 yr-old female with a PMHx of HTN and CKD III, presented with sub-sternal chest pain, palpitations, headaches and malignant hypertension. Exam was significant for BP 211/136 mm Hg, HR 84/m and trace LE edema. Laboratory data: Hb 10.6 g/dL, Sr Cr 2.5 mg/dL, and troponin, b-hCG and urine toxicology screen were negative. Urine microscopy did not reveal RBC casts; spot urine protein/Cr ratio was 1.14. TTE showed moderate pericardial effusion, and a pericardial window drained 100 mL of transudative fluid with benign mesothelial cells, and benign pericardial tissue on biopsy.
An ANA titer of 160 and low C4 < 8.0 mg/dL (C3 normal 93.9 mg/dL) increased suspicion for lupus nephritis. However, renal pathology revealed thrombotic microangiopathy, moderate to severe chronic sclerosing nephropathy, severe arteriolar hyalinosis, myxoid intimal hyperplasia, fibrin thrombi, suggestive of chronic thrombotic microangiopathy. IF did not show “full house” staining.
While the patient had no history of Raynaud’s phenomenon, or hallmark sclerotic skin changes of Systemic sclerosis (SSc), diagnosis of SSc was confirmed with positive scleroderma Ab (6.0); antiphospholipid Ab, and serum cryoglobulin were also positive. Beta blockers were discontinued to prevent vasospasm; ACE inhibitor and calcium channel blocker were initiated.

Conclusion

Prompt diagnosis is needed for timely initiation of ACE inhibition, which has dramatically improved renal outcomes. Hence it is crucial to recognize atypical clinical features of SSc such as pericardial effusion due to primary cardiac involvement, or secondary to pulmonary hypertension. Pericardial effusion may also be a surrogate for active or severe disease, signifying poor prognosis in cases with primary cardiac involvement.