scholarly journals Systemic sclerosis complicated with renal thrombotic microangiopathy: a case report and literature review

2022 ◽  
Vol 23 (1) ◽  
Author(s):  
Weiwei Kong ◽  
Yaomin Wang ◽  
Huiping Wang ◽  
Qin Zhou ◽  
Jianghua Chen ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Lupus Erythematosus ◽  
Thrombotic Microangiopathy ◽  
Connective Tissue Diseases ◽  
Kidney Injury ◽  
Renin Angiotensin System ◽  
Overlap Syndrome ◽  
Scleroderma Renal Crisis ◽  
Related Factors ◽  
Complex Deposition

Abstract Background Systemic sclerosis (SSc) may overlap with other connective tissue diseases, which is named overlap syndrome. Scleroderma renal crisis (SRC) is a rare but severe complication of SSc. SSc related thrombotic microangiopathy (SSc-TMA) is an infrequent pathology type of SRC, while SSc-TMA accompanied by overlap syndrome is very rare. Case presentation This study reported a case of acute kidney injury (AKI) accompanied with overlap syndrome of SSc, systemic lupus erythematosus (SLE) and polymyositis (PM). The renal pathology supported the diagnosis of SSc-TMA but not SLE or PM-related renal injury, characterized by renal arteriolar thrombosis, endothelial cells edema, little cast in tubules and mild immune complex deposition. The primary TMA related factors (ADAMTS13 and complement H factor) were normal. Thus, this case was diagnosed as secondary TMA associated with SSc. The patient was treated with renin angiotensin system inhibitors, sildenafil, supportive plasma exchange/dialysis, and rituximab combined with glucocorticoids. After 2 months of peritoneal dialysis treatment, her renal function recovered and dialysis was stopped. Conclusion This study presented a case of SSc-TMA with overlap syndrome. Rituximab can be used as a treatment option in patients with high SRC risk or already manifesting SRC.

scholarly journals Patients with Systemic Sclerosis with and without Overlap Syndrome Show Similar Microvascular Abnormalities

Diagnostics ◽  
2021 ◽  
Vol 11 (9) ◽  
pp. 1606
Author(s):  
Gabriella Nagy ◽  
László Czirják ◽  
Gábor Kumánovics
Keyword(s):  
Rheumatoid Arthritis ◽  
Systemic Sclerosis ◽  
Lupus Erythematosus ◽  
Connective Tissue Diseases ◽  
Capillary Density ◽  
Overlap Syndrome ◽  
Inflammatory Myopathies ◽  
Systemic Lupus ◽  
Control Groups ◽  
Morphology Parameters

Introduction: Nailfold video capillaroscopy (NVC) is a useful tool for measuring capillary density (CD) and capillary morphology parameters and is mainly used in systemic sclerosis (SSc). Objective: We aimed to assess the prevalence of an SSc pattern and CD in different connective tissue diseases (CTDs). Methods: NVC was performed on 268 patients with CTDs. Control groups consisted of 104 healthy volunteers (HVs) and 36 primary Raynaud’s patients (PRPs). Results: Decreased CD was more prevalent in SSc, systemic lupus erythematosus (SLE), inflammatory myopathies (IIM), and overlap CTD patients compared with both controls. Average CD, the prevalence of decreased CD, and the prevalence of an SSc pattern did not differ significantly between SSc patients with (n = 39) and without (n = 50) overlap syndrome. An SSc pattern was significantly more prevalent in SLE (23%), SSc (82%), IIM (35%), and rheumatoid arthritis (17%) compared with both control groups. The prevalence of an elevated microangiopathy evaluation score (MES) was significantly higher in SLE, SSc, and IIM than in the HVs. Conclusion: The presence of another CTD in SSc did not influence CD or morphology. An SSc pattern may also be present in CTDs other than SSc. The MES is a useful instrument to distinguish between patients with CTDs and controls.

scholarly journals Acute Kidney Injury Relevant to Tubulointerstitial Nephritis with Late-Onset Uveitis Superimposed by Thrombotic Microangiopathy: A Case Report and Review of the Literature

2020 ◽  
Vol 6 (6) ◽  
pp. 414-421
Author(s):  
Youlu Zhao ◽  
Junwen Huang ◽  
Tao Su ◽  
Zhikai Yang ◽  
Xizi Zheng ◽  
...  
Keyword(s):  
Acute Kidney Injury ◽  
Thrombotic Microangiopathy ◽  
Tubulointerstitial Nephritis ◽  
Late Onset ◽  
Drug Hypersensitivity ◽  
Oral Prednisone ◽  
Kidney Injury ◽  
Renin Angiotensin System ◽  
Renal Recovery

<b><i>Background:</i></b> The syndrome of tubulointerstitial nephritis and uveitis (TINU) is an uncommon and multisystemic autoimmune disorder. This review reports a rare case of TINU being superimposed on thrombotic microangiopathy (TMA) and, by comparing with the available literature, also summarizes the clinical features, associated conditions, treatment, and outcome of patients with TINU. <b><i>Summary:</i></b> Herein, we report the case of a 37-year-old male patient with acute kidney injury (AKI) clinicopathologically identified as malignant hypertension-induced TMA superimposed by acute tubulointerstitial nephritis, which was suspected to be related to drug hypersensitivity. After treatment with oral prednisone combined with a renin-angiotensin system inhibitor, the patient achieved partial renal recovery and was withdrawn from hemodialysis. Recurrent AKI concomitant with new-onset asymptomatic uveitis was detected during routine clinical follow-up after cessation of prednisone. TINU was then diagnosed, and prednisone followed by cyclophosphamide was prescribed. The patient achieved better renal recovery than in the first round of treatment and maintained stable renal function afterward. By reviewing the literature, 36 cases were reported as TINU superimposed on other conditions, including thyroiditis, osteoarthropathy, and sarcoid-like noncaseating granulomas. <b><i>Key messages:</i></b> TINU could be complicated by many other conditions, among which TMA is very rare. When presented as AKI, kidney biopsy is important for differential diagnosis. The case also shows that recurrent AKI with concomitant uveitis after prednisone withdrawal strongly suggested the need for long-term follow-up and elongated prednisone therapy for TINU syndrome.

scholarly journals OVERLAP SYNDROME OF SYSTEMIC LUPUS ERYTHEMATOSUS AND SYSTEMIC SCLEROSIS IN A HIV PATIENT: IMMUNODEFICIENCY AND AUTOIMMUNITY COMING TOGETHER

2019 ◽  
Author(s):  
GABRIEL PACÍFICO SEABRA NUNES ◽  
PATRÍCIA DA SILVA CUNHA NOEL ◽  
SIMONE FERREIRA DE CASTRO BOTILEIRO ◽  
SANDRA LÚCIA EUZÉBIO RIBEIRO
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Systemic Sclerosis ◽  
Lupus Erythematosus ◽  
Overlap Syndrome ◽  
Systemic Lupus

scholarly journals Antifibrillarin autoantibodies present in systemic sclerosis and other connective tissue diseases interact with similar epitopes.

1995 ◽  
Vol 181 (3) ◽  
pp. 1027-1036 ◽  
Author(s):  
K N Kasturi ◽  
A Hatakeyama ◽  
H Spiera ◽  
C A Bona
Keyword(s):  
Systemic Sclerosis ◽  
Connective Tissue ◽  
Lupus Erythematosus ◽  
Competitive Inhibition ◽  
Molecular Mimicry ◽  
Connective Tissue Diseases ◽  
Nuclear Antigen ◽  
Barr Virus ◽  
Recombinant Fusion Proteins ◽  
Epstein Barr

Autoantibodies specific against fibrillarin, a 34-kD nucleolar protein associated with U3-snRNP, are present in patients with systemic sclerosis (SSc). To understand the mechanisms involved in the induction of these autoantibodies, we prepared a series of human fibrillarin recombinant proteins covering the entire molecule and analyzed their interaction with the autoantibodies present in various connective tissue diseases. Our results showed that antifibrillarin autoantibodies are present not only in SSc, as previously reported, but also in a variety of other connective tissue diseases. Patients with SSc (58%), mixed connective tissue diseases (60%), CREST syndrome (calcinosis, Raynaud phenomenon, esophageal dismotility, sclerodactyly, and telangiectasia syndrome) (58%), systemic lupus erythematosus (39%), rheumatoid arthritis (60%), and Sjogern's syndrome (84%) showed presence of antifibrillarin autoantibodies. Results obtained from competitive inhibition radioimmunoassay and Western blot analyses with purified recombinant fusion proteins revealed that these autoantibodies react primarily with epitope(s) present in the NH2- (AA 1-80) and COOH-terminal (AA 276-321) domains of fibrillarin. Autoantibodies reacting with internal regions of fibrillarin are less frequent. Analysis of the hydrophilicity profiles of reactive peptides showed presence of three potential antigenic sites in the NH2- and two in the COOH-terminal regions. While a hexapeptide sequence NH2 terminus of fibrillarin is shared with an Epstein-Barr virus-encoded nuclear antigen, the COOH-terminal region shares sequence homology with P40, the capsid protein encoded by herpes virus type 1. Interestingly, these two regions of fibrillarin also contain the most immunodominant sequences, as predicted by surface probability and the Jameson and Wolf antigenic index. These observations suggest that molecular mimicry might play an important role in the induction of antifibrillarin autoantibodies.

Pulmonary hypertension on systemic sclerosis-lupus erythematosus overlap syndrome

2019 ◽  
Vol 68 (4) ◽  
pp. 221-225
Author(s):  
I. Rachdi ◽  
M. Tougorti ◽  
F. Daoud ◽  
Z. Aydi ◽  
H. Zoubeidi ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Lupus Erythematosus ◽  
Overlap Syndrome
Sign in / Sign up

Export Citation Format

Share Document