Scleroderma renal crisis can precede the diagnosis of SSc or be the presenting manifestation of the disease. Rapid clinical assessment of these patients is critical, looking for elevated blood pressure, acute kidney injury, signs of malignant hypertension and signs of thrombotic microangiopathies (i.e., hemolytic anemia, low platelets; also see https://www.the-rheumatologist.org/article/thrombotic-microangiopathies-rheumatology).
Confounding the picture is the fact that patients with renal crisis can present without renal failure or without hypertension. Dr. Boin stressed that performing a renal biopsy is key when renal crisis is suspected. This is important to confirm the diagnosis, rule out other entities and assess the extent of damage and potential for reversibility, all of which can inform prognosis.
Myocardial Disease
The final area of organ involvement that Dr. Boin discussed was myocardial involvement. This complication drives early mortality in SSc, with small vessel ischemia and myocardial inflammation. These patients may even experience sudden death, and thus an automated implantable defibrillator (AICD) can be lifesaving.
Patients at highest risk for the development of myocardial disease in SSc are those who are older, male, Black, and have a history of myositis and arthritis.
Jason Liebowitz, MD, is an assistant professor of medicine in the Division of Rheumatology at Columbia University Vagelos College of Physicians and Surgeons, New York.
References
- Jaeger VK, Wirz EG, Allanore Y, et al; EUSTAR co-authors. Incidences and risk factors of organ manifestations in the early course of systemic sclerosis: A longitudinal EUSTAR study. PLoS One. 2016 Oct 5;11(10):e0163894.
- Shah AA, Wigley FM. My approach to the treatment of scleroderma. Mayo Clin Proc. 2013 Apr;88(4):377–393.
- Bernstein EJ, Khanna D, Lederer DJ. Screening high-resolution computed tomography of the chest to detect interstitial lung disease in systemic sclerosis: A global survey of rheumatologists. Arthritis Rheumatol. 2018 Jun;70(6):971–972.
