Successful bosentan therapy in a case of pulmonary arterial hypertention developed during immunosuppressive therapy for lupus nephritis.

AbstractPosterior reversible encephalopathy syndrome is a rare manifestation of systemic lupus erythematosus, characterized by altered mental status, headache, convulsions, visual field impairment and posterior and reversible alterations on imaging scans(1,2). The clinical picture develops over a few hours, presenting with rapidly progressive neurological symptoms(3). It was first described in 1996. It is more frequent in patients with acute kidney injury or chronic kidney disease, thus in lupus patients with kidney disorders. It is associated with hypertension, other autoimmune diseases beside lupus, immunosuppressive therapies, especially antibody-based immunosuppressive therapy, and organ transplantation. It is clinically reversible within one week and imaging changes resolve within 2-4 weeks. It is treatable and has a good prognosis. We present the case of a young woman of 27 years, diagnosed with systemic lupus erythematosus who developed convulsive seizures, headache, visual impairment, being under immunosuppressive therapy with azathioprine. The kidney biopsy revealed class IV lupus nephritis and partial remission of the nephrotic syndrome. The other manifestations of SLE in this patient were cutaneous, immunological, articular and haematological. The patient had a good short, medium and long-term prognosis at 30 days and also at 6 months.

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Early-stage predictors for treatment responses in patients with active lupus nephritis

Lupus ◽

10.1177/0961203319826703 ◽

2019 ◽

Vol 28 (3) ◽

pp. 283-289 ◽

Cited By ~ 2

Author(s):

G Liu ◽

H Wang ◽

J Le ◽

L Lan ◽

Y Xu ◽

...

Keyword(s):

Lupus Nephritis ◽

Serum Albumin ◽

Immunosuppressive Therapy ◽

Early Stage ◽

Complement C3 ◽

Class Iii ◽

Class V ◽

Predictive Values ◽

Cutoff Value ◽

Active Lupus Nephritis

Objective We analyzed data of lupus nephritis (LN) patients to find parameters that can predict remission. Methods Sixty-four LN patients who were diagnosed with class III, IV, V or V + III/IV by renal biopsy and were followed up for more than six months in our center were enrolled retrospectively. Receiver operating characteristic curves were used to test the predictive values of urinary protein-to-creatinine ratio (UPCR), serum albumin and complement C3 at the first, second and third months as predictors for remission at the sixth month. Results The patients' renal pathologies were class III (five cases), class IV (33 cases), class V (nine cases) and class V + III/IV (17 cases). All patients received standard immunosuppressive therapy. Forty-six (71.9%) patients (grouped as the remission group) achieved remission at the end of the sixth month, including 23 complete remissions and 23 partial remissions. The other 18 patients were grouped as the no-remission group. There were no significant differences in clinical data, proportion of immunosuppressive therapy or renal pathological characteristics between the remission group and no-remission group at baseline, except the serum urea nitrogen of the remission group was lower than in the no-remission group. The UPCR were significantly lower in the remission group than in the no-remission group at months 1, 2, 3 and 6, respectively, while the serum albumin was significantly higher in the remission group than in the no-remission group at months 3 and 6, respectively. There were no significant differences in serum creatinine between the remission group and no-remission group, except at month 1. The C3 levels were higher in the remission group than in the no-remission group at months 1, 2 and 3, respectively. The areas under the curve (AUC) of the change percentage of UPCR at month 3 and the serum albumin at month 3 were the most significant (AUC 0.778, p = 0.002; AUC 0.773, p = 0.001, respectively). The cutoff value of the change percentage of UPCR at month 3 was 59%. The cutoff value of serum albumin at month 3 was 32.9g/l. Conclusion The change percentage of UPCR ≥59% and the serum albumin ≥32.9 g/l at the third month were valuable for predicting remission at the sixth month in LN. Because of the small-size and retrospective nature, this study needs to be validated.

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263 Time of response to immunosuppressive therapy for lupus nephritis patients

10.1136/lupus-2019-lsm.263 ◽

2019 ◽

Author(s):

Natalia Rivera ◽

Esther Ruiz ◽

M Luz García ◽

Eva Galindez ◽

Olaia Fernández ◽

...

Keyword(s):

Lupus Nephritis ◽

Immunosuppressive Therapy

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Management and long-term outcomes of sarcoidosis-associated pulmonary hypertension

European Respiratory Journal ◽

10.1183/13993003.00465-2017 ◽

2017 ◽

Vol 50 (4) ◽

pp. 1700465 ◽

Cited By ~ 43

Author(s):

Athénaïs Boucly ◽

Vincent Cottin ◽

Hilario Nunes ◽

Xavier Jaïs ◽

Abdelatif Tazi ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Targeted Therapy ◽

Immunosuppressive Therapy ◽

Exercise Capacity ◽

Stage Iv ◽

Long Term Outcomes ◽

Hypertension Diagnosis ◽

Pulmonary Arterial

Studies reporting the effects of modern strategies with pulmonary arterial hypertension (PAH)-targeted therapies in sarcoidosis-associated pulmonary hypertension (S-APH) are limited.Clinical and haemodynamic data from newly diagnosed patients with severe S-APH (mean pulmonary artery pressure (mPAP) >35 mmHg or mPAP 25–35 mmHg with cardiac index <2.5 L·min−1·m−2) were collected from the French Pulmonary Hypertension Registry between 2004 and 2015.Data from 126 patients with severe S-APH were analysed (mean±sd age 57.5±11.6 years, 74% radiological stage IV). 97 patients (77%) received PAH-targeted therapy and immunosuppressive therapy was initiated or escalated in 33 patients at the time of pulmonary hypertension diagnosis. Four months after PAH-targeted therapy initiation, mean±sd pulmonary vascular resistance decreased from 9.7±4.4 to 6.9±3.0 Wood units (p<0.001), without significant improvement in exercise capacity. Among the 11 patients treated only with immunosuppressive therapy, a haemodynamic improvement was observed in four patients, including two with compressive lymph nodes. After a median follow-up of 28 months, 39 patients needed PAH-targeted therapy escalation, nine underwent lung transplantation and 42 had died. Survival at 1, 3 and 5 years was 93%, 74% and 55%, respectively.PAH-targeted therapy improved short-term pulmonary haemodynamics in severe S-APH without change in exercise capacity. Immunosuppressive therapy improved haemodynamics in selected patients. Pulmonary hypertension in sarcoidosis remains associated with a poor prognosis.

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Systemic Sclerosis and Systemic Lupus Erythematosus Overlap Syndrome with Pulmonary Arterial Hypertension Successfully Treated with Immunosuppressive Therapy and Riociguat

Cureus ◽

10.7759/cureus.4327 ◽

2019 ◽

Cited By ~ 1

Author(s):

Kentaro Kuzuya ◽

Soichiro Tsuji ◽

Masato Matsushita ◽

Shiro Ohshima ◽

Yukihiko Saeki

Keyword(s):

Systemic Lupus Erythematosus ◽

Systemic Sclerosis ◽

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Immunosuppressive Therapy ◽

Lupus Erythematosus ◽

Overlap Syndrome ◽

Systemic Lupus ◽

Pulmonary Arterial

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P1792SUCCESSFUL TRANSPLANTATION OF STEM CELLS AND KIDNEY IN REFRACTORY LUPUS NEPHRITIS: REMOTE OUTCOMES

Nephrology Dialysis Transplantation ◽

10.1093/ndt/gfaa142.p1792 ◽

2020 ◽

Vol 35 (Supplement_3) ◽

Author(s):

Aleksandr Demin ◽

Larisa Demina

Keyword(s):

Stem Cells ◽

Lupus Nephritis ◽

Creatinine Clearance ◽

Disease Activity ◽

Immunosuppressive Therapy ◽

High Dose ◽

Sledai Score ◽

Organ Systems ◽

Nephritic Syndrome ◽

Conventional Immunosuppression

Abstract Background and Aims Lupus nephritis is a common part of systemic lupus erythematosus (SLE), severe autoimmune disease that affects multiple organ systems and associated with pure prognosis. Patients with lupus nephritis who experience persisted disease activity despite conventional immunosuppression are at high risk of early death. Re-setting of the immune system and self-tolerance by high-dose immunosuppressive therapy with autologous stem cell transplantation (ASCT) is a new approach in the treatment of refractory SLE. Remote outcomes of this method and effectiveness relapse treatment are still unclear and were the aims of this study. Method We report a sick woman 39 years old, with refractory severe lupus nephritis. Standard therapy was ineffective, SLEDAI score remains 22 so patient was underwent high dose immunosuppressive therapy with ASCT and included in European Group for Blood and Marrow Transplantation European League against Rheumatism (EBMT/EULAR) registry on 53 pts who received ASCT for SLE between 1996 and 2005. Ethical approval was obtained for this study from Cambridge University Hospital Ethics Committee. Results The ASCT induced complete clinical and serological remission (SLEDAI score was 0) for 3 years. Than relapse with nephritic syndrome and anti-dsDNA positivity occurred and caused renal failure, creatinine clearance decreased to 21. Despite therapy including prednisolone, 45 mg daily, and mycophenolate mofetil, 2000 mg daily, disease activity persisted, creatinine clearance remained decreasing and 3 years later became 15 ml/min. During 1 year patient was on regular hemodialysis, then renal transplantation performed. Now, follow up is 15 years after ASCT and 8 years after kidney transplantation. Patient received a standard post-transplant immunosuppression with prednisolone, 5 mg daily, tacrolimus, 2.5 mg daily, and azathioprine, 100 mg daily, and her conditions remains stable, she has functioning renal allograft, SLEDAI score is 2 (anti-dsDNA positivity in low titer). Conclusion We present the successful remote outcomes of first case of immunoablation and double transplantation of autologous stem cells and allogeneic kidney in severe refractory to conventional immunosuppression SLE with lupus nephritis.

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