Scleroderma Renal Crisis Associated With Microangiopathic Hemolytic Anemia in a Patient With Seronegative Scleroderma and Monoclonal Gammopathy

Abstract

Systemic sclerosis with negative serology, particularly that complicated by scleroderma renal crisis (SRC), is rarely encountered. We describe a patient with seronegative systemic sclerosis who developed acute kidney injury, proteinuria, and hypertensive emergency following motor vehicle-related trauma and in the setting of nonsteroidal anti-inflammatory drug use. Findings on physical examination, imaging, and skin biopsy led to a clinical diagnosis of scleroderma, despite the lack of supportive laboratory data. IgG lambda paraproteinemia was detected on workup. Bone marrow biopsy showed plasmacytosis and trace lambda-restricted plasma cells consistent with monoclonal gammopathy of undetermined significance. Chemotherapy was initially started given concern for myeloma with cast nephropathy but was later stopped after a kidney biopsy revealed thrombotic microangiopathy (TMA). The SRC associated with TMA was ultimately diagnosed, though atypical hemolytic uremic syndrome (aHUS) induced perhaps by monoclonal gammopathy or hypertension was also possible. Captopril and eculizumab were initiated for SRC and aHUS, respectively. Despite therapy, renal function did not recover, and the patient required hemodialysis indefinitely. This case highlights clinical features common to both SRC and aHUS as well as points out a few ways to differentiate between them.

Double Hit of Hydroxichloroquine and Amiodarone Induced Renal Phospholipidosis in a Patient with Monoclonal Gammopathy and Sclerodermiform Syndrome: A Case Report and Review of the Literature

“…Our view is that the fact that the patient had SS is not related to her renal involvement or to the development of renal PLD. First, our patient was diagnosed with SS with positivity for anti-centromere antibodies and ANA, which is less related to renal involvement; there was no involvement of other organs such as the lungs or heart; and there was no suspicion of scleroderma renal crisis (SRC) in the absence of hypertension and vascular involvement in the RB [ 21 ]. We could not to find any association between DIP, SS and GMSR in the literature, so we think that their relationship has a casual rather than causal effect.…”

Thrombotic microangiopathy after traumatic brain injury: A case report and review of the literature

“…Trauma, however, is not included in the classic etiology in many reviews and has yet only recently been recognized as a potential mechanism of secondary TMA. 4 We could only identify three case reports handling TMA following major trauma [5][6][7] The first case described TMA following blunt abdominal liver injury in a 23 yearold male who suffered a motor vehicle accident. The diagnosis was only made after 21 days and following treatment for sepsis and start of hemodialysis, 6 which is longer and more complicated than in our case.…”

“…The time frame of the case is consistent with our case, but an important difference is that our patient had a sustained diuresis during the hospitalization 5 . More recently, Riley et al described microangiopathic hemolytic anemia in a patient following a motor vehicle accident, but was in further work‐up diagnosed with scleroderma based on findings on the kidney biopsy, which can also be a cause of TMA 7 …”

“…5 More recently, Riley et al described microangiopathic hemolytic anemia in a patient following a motor vehicle accident, but was in further work-up diagnosed with scleroderma based on findings on the kidney biopsy, which can also be a cause of TMA. 7 Further, "probable" TMA following trauma (t-TMA) was described in a retrospective single-center case series of patients admitted to the ICU between 2018 and 2019. Of 1164 screened patients 20 (1.7%) were categorized as having t-TMA by meeting several diagnostic criteria: having a recent trauma, having transfusion refractory thrombocytopenia, laboratory abnormalities suggestive of TMA, and finally exclusion of other causes of TMA.…”

“…Recently, trauma has been suggested as a trigger for initiating TMA. 4 Progression of TIC to TMA, however, has not been described and only a few case reports [5][6][7] and one case series 8 describe TMA following trauma. We report an additional patient with TMA following an isolated traumatic brain injury.…”