Scleroderma renal crisis as initial presentation of scleroderma

Sahil Bagai; Scienthia Sanjeevani; Dinesh Khullar; Bhavna Bansal; Vipra Malik

doi:10.4103/injms.injms_123_21

Users Online: 122

CASE REPORT

Year : 2022 | Volume : 13 | Issue : 2 | Page : 122-123

Scleroderma renal crisis as initial presentation of scleroderma

Sahil Bagai¹, Scienthia Sanjeevani¹, Dinesh Khullar¹, Bhavna Bansal², Vipra Malik³
¹ Department of Nephrology and Renal Transplant Medicine, Max Superspeciality Hospital, Saket, Delhi, India
² Department of Pathology, Max Superspeciality Hospital, Saket, Delhi, India
³ Department of Pathology, Core Diagnostics Pvt Ltd, Gurugram, Haryana, India

Date of Submission	20-Oct-2021
Date of Decision	14-Dec-2021
Date of Acceptance	20-Dec-2021
Date of Web Publication	21-Mar-2022

Correspondence Address:
Dr. Sahil Bagai
Consultant Department of Nephrology and Renal Transplant Medicine, Max Superspeciality Hospital, Saket, Delhi
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/injms.injms_123_21

Abstract

Scleroderma renal crisis (SRC) is characterized by the development of severe or worsening arterial hypertension associated with the abrupt onset of progressive renal failure in the absence of any other cause. Only 1% of limited and 4–11% of diffuse scleroderma cases have SRC. We report a case of SRC as an initial presentation in a newly diagnosed case of scleroderma.

Keywords: Scleroderma renal crisis, systemic sclerosis, thrombotic microangiopathy

How to cite this article:
Bagai S, Sanjeevani S, Khullar D, Bansal B, Malik V. Scleroderma renal crisis as initial presentation of scleroderma. Indian J Med Spec 2022;13:122-3

How to cite this URL:
Bagai S, Sanjeevani S, Khullar D, Bansal B, Malik V. Scleroderma renal crisis as initial presentation of scleroderma. Indian J Med Spec [serial online] 2022 [cited 2023 Mar 31];13:122-3. Available from: http://www.ijms.in/text.asp?2022/13/2/122/339996

Introduction

Scleroderma renal crisis (SRC) is a life threatening complication of systemic sclerosis which presents with malignant hypertension and decreased urine output and has mortality around 10%.^[1],[2] It is mostly seen in patients with diffuse scleroderma. Majority of cases happen during the initial 4 years of illness, and median time of onset is averagely 8 months from the onset of disease. In about one-fifth of all cases of systemic sclerosis (SSc), SRC is the first presentation of the disease, and subset of these patients has no skin involvement.^[3]

Case Report

In our case, a known elderly diabetic and treated case of carcinoma breast presented to the emergency room (ER) with swelling feet and palpitations accompanied by decreased urine output for 3 days. She denied any recent history of fever, rash, or drug intake. On examination, she had blood pressure of 200/120 mmHg in right arm supine, pulse rate of 100/min, pitting pedal edema, and raised jugular venous pressure. No skin or nail fold changes were present upon arrival in ER. Fundus examination revealed moderate nonproliferative diabetic retinopathy bilaterally with the absence of any macular changes. On systemic examination, she had bilateral coarse crepitations in the infrascapular area. Laboratory analysis revealed hemoglobin of 7.2 g/dl, total leukocyte count 6200/μl, platelets of 1, 50,000/μl, and erythrocyte sedimentation rate of 135. Peripheral smear showed occasional schistocytes. Hemolytic work up showed low serum haptoglobin level (<30 mg/dl), corrected reticulocyte count 7.12, and a negative direct Coombs test. Renal function revealed serum creatinine of 3.5 mg/dl and blood urea 66 mg/dl. Other biochemical parameters were normal. Iron profile was suggestive of iron-deficiency anemia. Urine routine microscopy revealed 1 + protein, 7–10 red blood cells/high-power field (HPF), 5–25 pus cells/HPF, and urine spot protein creatinine ratio was 3.84 mg/g. Antinuclear antibodies by immunofluorescence showed strong positivity with titers of 1:2560 exhibiting nucleolar/speckled pattern. The extended autoimmune profile revealed antipolymyositis/Scl antibodies (3+). Nail fold capillaroscopy showed multiple giant capillaries and suggestive of Raynaud's phenomenon. Ultrasound abdomen did show bilateral normal kidneys with cortical thickness around 1cm. Chest X-ray showed blunting of bilateral costophrenic angles with some honeycombing seen in both lower lobes. With presumptive clinical diagnosis of SRC, she underwent a percutaneous kidney biopsy. Light microscopy showed endothelial swelling with mesangiolysis, wrinkled basement membrane, and fibrin thrombi in arterioles seen in silver stain, consistent with the diagnosis of thrombotic microangiopathy (TMA), and immunofluorescence was negative [Figure 1]a and [Figure 1]b. She was started on angiotensin-converting enzyme inhibitor (ACEI), ramipril and mycophenolate mofetil for SRC. Complement factors and a disintegrin and metalloproteinase with thrombospondin motifs 13 (ADAMTS 13) levels were not done due to nonavailability of tests at our center. Therapeutic plasma exchange (TPE) was also being considered, but her platelet counts and hemoglobin started stabilizing and normalized within the next 5 days. She had a persistent rise in creatinine with the use of ACEI and had to be dialyzed transiently for about 2 weeks and then had recovery in renal function and dialysis was stopped. Last follow-up available is 1-year postillness, she continues to have serum creatinine around 2.0 mg/dl and stable platelet and hemoglobin counts.

Figure 1: (a) Glomerular vascular pole shows luminal occlusion by endothelial cell swelling (black arrow). Furthermore, seen are small vessels/arterioles with luminal occlusion by severe intimal edema and endothelial cell swelling (PAS stain, ×10) (b) Fibrin thrombi seen in glomerular capillary loops (black arrow) and arterioles (H and E)

Click here to view

Discussion

Renal involvement is common in SSc, and SRC is the one with most dramatic clinical course. Vasculopathy and immune dysregulation play a pivotal role in the pathogenesis of the disease. Antineutrophil cytoplasmic antibody-associated vasculitis, an isolated reduced glomerular filtration rate in SSc, and antiphospholipid-associated nephropathy are some other forms of renal injury seen in a case of SSc.^[4] Risk factors for SRC include diffuse skin involvement, the presence of anti-RNA polymerase III antibodies, the use of corticosteroids in doses >15 mg a day, tendon friction rubs, new-onset anemia, pericarditis, and congestive heart failure which were not present in our case.^[5]

Histopathology findings in our case were consistent with SRC changes reported in the literature.^[6],[7] In acute stages, glomeruli usually show ischemic collapse or fibrinoid necrosis and the tubules show tubular degeneration and necrosis, whereas in the chronic stages, tubular atrophy and interstitial fibrosis develops.

ACEIs form the backbone of the management of SRC. ACEIs have changed the outcome of patients with SRC. One-year survival rate of patients without ACEI is around 10%, whereas patients treated with ACEI have about 85% survival at 1 year and 65% survival at 5 years.^[8] In certain cases, TPE is required when TMA is associated with the absence of ADAMTS 13 enzyme. In our case, we had considered TPE as option as ADAMTS 13 testing was not available but was not required as the patient had improvement seen on addition of ACEI.

Prognosis in SRC with the use of ACEI has improved drastically. There are various factors in SRC associated with poor outcomes such as serum creatinine >3 mg/dl at presentation, delay in initiating antihypertensive treatment, older age, and renal biopsy findings showing arteriolar fibrinoid necrosis, severe glomerular ischemic collapse.^[5] In our case, even though the patient had factors favoring a poor outcome but patient after initial obstacles managed to have renal recovery.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

None.

Conflicts of interest

There are no conflicts of interest.

References

1.	Mouthon L, Bussone G, Berezné A, Noël LH, Guillevin L. Scleroderma renal crisis. J Rheumatol 2014;41:1040-8.
2.	Howell CM, Juakiem W. Scleroderma renal crisis; presentation, treatment and prognosis. Consultant 2016;56:46-50.
3.	Logee KM, Lakshminarayanan S. Scleroderma renal crisis as an initial presentation of systemic sclerosis: A case report and review of the literature. Clin Exp Rheumatol 2015;33:S171-4.
4.	Chrabaszcz M, Małyszko J, Sikora M, Alda-Malicka R, Stochmal A, Matuszkiewicz-Rowinska J, et al. Renal involvement in systemic sclerosis: An update. Kidney Blood Press Res 2020;45:532-48.
5.	Steen VD. Kidney involvement in systemic sclerosis. Presse Med 2014;43:e305-14.
6.	Guillevin L, Mouthon L. Scleroderma renal crisis. Rheum Dis Clin North Am 2015;41:475-88.
7.	George JN. How i treat patients with thrombotic thrombocytopenic purpura: 2010. Blood 2010;116:4060-9.
8.	Penn H, Howie AJ, Kingdon EJ, Bunn CC, Stratton RJ, Black CM, et al. Scleroderma renal crisis: Patient characteristics and long-term outcomes. QJM 2007;100:485-94.