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Year : 2021  |  Volume : 64  |  Issue : 1  |  Page : 217-219
Sudden renal failure in a case of overlap connective tissue disorder—A diagnostic dilemma

1 Department of Pathology, IPGMER and SSKM Hospital, Kolkata, West Bengal, India
2 Department of Nephrology, IPGMER and SSKM Hospital, Kolkata, West Bengal, India
3 Department of Anatomy, IPGMER and SSKM Hospital, Kolkata, West Bengal, India

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Date of Submission07-Oct-2019
Date of Decision13-Nov-2019
Date of Acceptance21-Jan-2020
Date of Web Publication8-Jan-2021

How to cite this article:
Jain K, Sengupta M, Basu K, Chowdhury AR, Bandopadhyay M. Sudden renal failure in a case of overlap connective tissue disorder—A diagnostic dilemma. Indian J Pathol Microbiol 2021;64:217-9

How to cite this URL:
Jain K, Sengupta M, Basu K, Chowdhury AR, Bandopadhyay M. Sudden renal failure in a case of overlap connective tissue disorder—A diagnostic dilemma. Indian J Pathol Microbiol [serial online] 2021 [cited 2022 Jan 19];64:217-9. Available from: https://www.ijpmonline.org/text.asp?2021/64/1/217/306532

Dear Editor,

Overlap connective tissue disorder is defined as a heterogeneous group in which two or more diseases occur simultaneously or in succession. Systemic lupus erythematosus (SLE) and systemic sclerosis (SSc) commonly present together with an average incidence of 14.8%.[1]As acute renal failure can be precipitated by both disorders, it is crucial to know the exact underlying etiology and thus tailor specific management.[2] Corticosteroids, which is recommended in lupus nephritis, is known to precipitate scleroderma renal crisis.

A 23-year-old female was admitted to the department of Nephrology with complaints of anuria, rapidly progressive dyspnea, and fever for the last 7 days. On examination, the patient was found to have a pulse rate of 118/min, BP-150/100 mm Hg, and anasarca. Serum creatinine was 6.58, albumin was 2.7. Urine routine examination (r/e) showed protein 3+, plenty of WBCs/high-power field (hpf), no casts or crystals. Serology was nonreactive. She had visited the rheumatology outdoor of our hospital 14 months ago with complaints of intermittent fever, fatigue, malar rash, and photosensitivity. She also had painful small joints and complained of bluish discoloration of fingers and toes during bathing in cold pond water. Routine workup was done and urine showed no sediments or evidence of proteinuria. Antibody panel was advised and showed U1 ribonucleoprotein (U1RNP) +, antinuclear antibody (ANA)+++, and anti scl 70 positivity. She was advised biopsy but refused. A provisional diagnosis of systemic lupus erythematosus was made, and she was started on hydroxychloroquine and nonsteroidal antiinflammatory drugs (NSAIDS) for arthralgia to which she responded well. She went to a local doctor 3 days prior to her admission where her urine r/e showed nephrotic range proteinuria and he suspected it to be a case of lupus nephritis and started her on steroids following which her symptoms worsened, and she was brought to our emergency. A working diagnosis of accelerated hypertension with lupus nephritis was made. She was continued on steroids and her condition worsened and had to be started on hemodialysis.

This time her renal biopsy with urgent reporting was done. A single core of renal tissue showing 18 glomeruli was obtained. One of the glomeruli was globally sclerosed and another one was segmentally sclerosed. The rests of the glomeruli along with the nonsclerosed tuft revealed wrinkling and thickening of the glomerular basement membrane and focal double contouring. Fibrinoid necrosis of afferent arteriole was identified in one glomerulus [Figure 1]. The vascular compartment showed myxoid intimal thickening along with myointimal cellular proliferation of interlobular arteries. Arterioles showed the evidence of thrombus along with fibrin insulation. Minimal (5%) interstitial fibrosis and tubular atrophy was found. Immunofluorescence study revealed trace C3 and C1q mesangial deposits. Considering the histological and immunofluorescence findings, a diagnosis suggestive of scleroderma renal crisis (SRC) was made.
Figure 1: (a): Prominent arterial onion skin lesion (arrows). (silver methanamine stain, 100 ×). (b) Glomerular mesangial RBCs (H and E stain, 400 ×). (c) afferent arteriole fibrinoid necrosis (Masson trichrome stain, 100 ×). (d) pin-point lumen of arterioles. (silver methanamine stain, 400 ×)

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Repeat serological tests showed Anti RO, Anti LA, ANA, anti-ds DNA, anti scl 70, and U1 ribonucleo protein (U1RNP) positivity. The case was revised and the patient was diagnosed as a case of scleroderma renal crisis in a case of mixed connective tissue disorder and started on angiotensin converting enzyme (ACE) inhibitors (Enalapril) and antihypertensives. Her vitals and clinical conditions started improving, and she was discharged in a stable condition. On follow-up, the patient had maintained a stable kidney function.

Scleroderma renal crisis is characterized by accelerated hypertension along with acute renal failure. Patients with diffuse cutaneous systemic sclerosis and rapidly progressive skin disease have been seen to predispose to SRC. Pericardial effusion, cardiac insufficiency, and new cardiac events are considered as imminent risk factors in the development of SRC.[3]

As per literature, a confirmatory diagnosis of SRC cannot be made without renal biopsy unless the patient has full-blown typical clinical features without any other overlapping CTDs.[4] Primary small vessel changes predominate over glomerular changes in SRC.[5] Early vascular changes manifest as myxoid material accumulation in the intima, thrombosis and/or fibrinoid necrosis. Onion skin-like lesion due to fibro intimal sclerosis with or without adventitial fibrosis is a manifestation of ongoing damage. A significant increase in arterial fibro intimal thickening was noted in SSC patients even in the absence of SRC and thus help in early diagnosis.

There is a spectrum of disorders, which presents with microangiopathic hemolytic anemia, which includes thrombotic thrombocytopenic purpura (TTP), hemolytic uremic syndrome (HUS), scleroderma renal crisis (SRC), antiphospholipid antibody syndrome (APS), and preeclampsia[6] Hence, differentiating them is of extreme importance for the commencement of treatment. APLA usually presents in a postpartum female, whereas atypical HUS is more prevalent in childhood than adulthood. Ancillary investigations like low level of a disintegrin and metalloprotease with thrombospondin type I domain 13 (ADAMTS13) in thrombotic thrombocytopenic purpura (TTP), high titers of anti-Ro and La antibody in SRC, and abnormalities of complement alternative pathway in atypical hemolytic uremic syndrome (HUS) helps to confirm the diagnosis.

An early accurate diagnosis helps in the aggressive and timely commencement of angiotensin converting enzyme inhibitors (ACEi), which improves renal outcome as well as leads to regression of dermatological manifestations. But around 40% of cases may still require dialysis as advanced tissue destruction may occur long before the diagnosis is made.[7] Due to defective immunity and immunosuppressive medication, patients may acquire tuberculosis infection.[8] Sterile pyuria may be the presenting features.[9]

Renal biopsy specimens with adequate numbers of glomeruli and vessels of all calibers are generally useful to identify aforesaid histopathological changes and thus help to establish a definite diagnosis.

Declaration of patient consent

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

Financial support and sponsorship

Department of Nephrology.

Conflicts of interest

There are no conflicts of interest.

   References Top

Iaccarino L, Gatto M, Bettio S, Caso F, Rampudda M, Zen M, et al.Overlap connective tissue disease syndromes. Autoimmun Rev 2013;12:363-73.  Back to cited text no. 1
Lockshin MD, Levine AB, Arkan D. Patients with overlapautoimmune disease differ from those with 'pure' disease.Lupus Sci Med 2015;2:e000084.  Back to cited text no. 2
Batal I, Domsic RT, Medsger TA, BastackyS.Scleroderma renal crisis: A pathology perspective. Int J Rheumatol2010;2010:543704.  Back to cited text no. 3
Lewandowski B, Domyslawska I, Klimiuk PA, Sierakowski S. Kidney crisis in systemic sclerosis. RoczAkad Med Bialymst 2005;50(Suppl 1):294-6.  Back to cited text no. 4
Batal I, Domsic RT, Shafer A, Medsger TA Jr, Kiss LP, Randhawa P, et al. Renal biopsy findings predicting outcome in scleroderma renal crisis. Hum Pathol2009;40:332-40.  Back to cited text no. 5
Abudiab M, Krause ML, Fidler ME, Nath KA, Norby SM. Differentiatingscleroderma renal crisis from other causes of thrombotic microangiopathy in a postpartum patient. Clin Nephrol 2013;80:293-7.  Back to cited text no. 6
Steen VD. Scleroderma renal crisis. Rheum Dis Clin North Am 2003;29:315-33.  Back to cited text no. 7
Ou SM, Fan WC, Cho KT, Yeh CM, Su? VYF, Hung MH, et al. Systemic sclerosis & the risk of tuberculosis. J Rheumatol 2014;41:1662-9.  Back to cited text no. 8
Jagtap SV, Nikumbh DB, Kanetkar S, Agarwal R, Khatib V.Classical renal tuberculosis presented as recurrent sterile pyuria and end stage kidney. Int J Health Sci Res 2012:2;127-32.  Back to cited text no. 9

Correspondence Address:
Moumita Sengupta
244 AJC Bose Road, Kolkata - 700 020, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJPM.IJPM_740_19

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