Treatment of Lupus Interstitial Lung Disease with Intravenous Cyclophosphamide

Eiser, Arnold R.; Shanies, Harvey M.

doi:10.1002/art.1780370318

Cited by 60 publications

(21 citation statements)

References 16 publications

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“…Although early studies cast doubts about the role of cytotoxic drugs in the treatment of lupus nephritis [20][21][22], the efficacy of pulse cyclophosphamide in the treat ment of renal and extrarenal complications of SLE has been shown [5,[8][9][10][11][12][13][14][15][16], Even though it is a short-term observation, our data also support these conclusions, studying patients with severe, steroid-resistant SLE. Con cerning the renal function, a failure rate of 20% was not unexpected for patients with severe SLE.…”

Section: Discussionsupporting

confidence: 73%

“…The optimal treatment for patients with SLE is not yet defined: corticosteroids have been the initial drug of choice, however, they are not effective in patients with the severe forms of this disease and may predispose to infections and late complications [1][2][3][4], Immunosuppressive drugs have been shown to be more effective than steroids alone in some patients [5][6][7][8]: the intermittent use of intravenous cyclophosphamide in the treatment of renal and extrarenal complications appears to be associated with clinical improvement of the SLE and stability of renal function [9][10][11][12][13][14][15][16]. There are, however, still some concerns about its effcctivity and toxicity in severe SLE [ 17], We report the results of an open trial using intravenous cyclophosphamide, intermittently, in the treatment of pa tients with severe SLE.…”

Section: Introductionmentioning

confidence: 99%

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Clinical Effects of Intermittent, Intravenous Cyclophosphamide in Severe Systemic Lupus erythematosus

Martinelli¹,

Pereira²,

Santos³

et al. 1996

Nephron

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To evaluate the clinical effectiveness of intermittent intravenous cyclophosphamide in the treatment of severe systemic lupus erythematosus, 20 patients with systemic lupus erythematosus (SLE) and evidence of severe renal involvement or systemic vasculitis, consecutively admitted to the hospital were studied. Cyclophosphamide was administered intravenously at a dosage of 1.0 g/m² monthly, during 6 months and maintained every 3 months during 12 additional months. Of 10 patients with active lupus nephritis, a reduction or disappearance of proteinuria and maintenance of normal renal function was recorded in 6. Improvement of renal function was observed in 4 out of 7 patients with renal insufficiency at initial evaluation; resolution of renal insufficiency was more frequently observed in patients with recent onset renal failure. At the end of the follow-up (18.0 ± 14.5 months) disappearance or reduction of nephrotic range proteinuria was recorded in 6 out of 14 patients; there was progression toward renal failure in 4 patients (20%). Response to intravenous cyclophosphamide therapy was observed in 4 of 5 patients with severe extrarenal SLE. Side effects, recorded in 12 patients, were mild and transient and in no patient was the treatment discontinued. Four patients died during the follow-up, although in 2 of them the deaths were not attributable to therapy. Even though this was an open and uncontrolled study, intermittent, intravenous cyclophosphamide was an effective therapy for severe, steroid refractory SLE.

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Section: Discussionsupporting

confidence: 73%

Section: Introductionmentioning

confidence: 99%

Clinical Effects of Intermittent, Intravenous Cyclophosphamide in Severe Systemic Lupus erythematosus

Martinelli¹,

Pereira²,

Santos³

et al. 1996

Nephron

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“…[3][4][5] Recently, the availability of intravenous cyclophosphamide (CYC) pulse therapy for CVD-ILD has been sporadically described. 1,4,6 We also reported a case that needed oral intubation with mechanical ventilation due to ILD associated with DM, and the respiratory distress condition was ameliorated by intravenous CYC pulse therapy. 7 However, the standard protocol concerning the dosage and the interval of CYC pulse therapy has not yet been established.…”

Section: Introductionmentioning

confidence: 91%

Intermittent intravenous cyclophosphamide pulse therapy for the treatment of active interstitial lung disease associated with collagen vascular diseases

et al. 2007

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The availability of intravenous cyclophosphamide (CYC) pulse therapy for collagen vascular diseases (CVD)-associated interstitial lung disease (ILD) has been indicated. However, the standard protocol concerning the dosage and the interval of CYC infusion has not yet been established. The aim of this study is to elucidate the efficacy and the safety of our "divided administration" protocol of CYC for the treatment of CVD-ILD. The treatment protocol consists of two steps: step 1, CYC 400-500 mg at 10-day intervals for at least 30 days, and step 2, CYC 500 mg at 14-day intervals for at least 4 weeks. The ILD activities were monitored by respiratory symptoms, serum levels of KL-6 (a serological marker of IP), chest computed tomography (CT), and pulmonary function tests. Seventeen patients [nonspecific interstitial pneumonia (NSIP), 12 patients; usual interstitial pneumonia (UIP), 4; lymphocytic interstitial pneumonia (LIP), 1] accomplished the study protocol. The sessions of CYC infusion ranged from 5 to 20 (mean, 8.3). In all patients, respiratory symptoms were improved and the serum levels of KL-6 were decreased (from 1572 +/- 904 to 978 +/- 392 U/ml; P < 0.01). Chest CT findings were improved in 4 patients (23.5%): they were all classified as NSIP; not deteriorated, 13 patients (76.5%). An improvement in the vital capacity percentage (%VC) was recognized in 10 patients (78.6%) and in diffusing capacity of carbon monoxide (%DLco) in 8 patients (61.5%). Nevertheless, mean %VC and mean %DLco did not change significantly. No major adverse event(s) occurred. The efficacy and safety of our "divided administration" protocol of CYC for CVD-ILD was demonstrated.

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“…When clinical or radiographic changes are slow or patients fail to respond to steroid therapy or relapse on steroid dose tapering, the addition of a steroid sparing agent, such as azathioprine, may be considered (14). Intravenous pulse cyclophosphamide combined with systemic corticosteroids has also been used successfully (15). In chronic, unremitting lupus pleurisy, refractory to medical therapy, pleurectomy, talc poudrage and tetracycline pleurodesis may be used.…”

Section: Case Reportmentioning

confidence: 99%

Systemic lupus erythematosus presenting as fulminant lupus pneumonitis: a rare case report

et al. 2016

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summaryWe report a case of 19 year-old female patient diagnosed as systemic lupus erythematosus (SLE) presented with fever and diffuse cutaneous lesions. During the hospital stay she had acute pneumonia, pleural effusion and respiratory failure, which required intensive care unit (ICU) care and mechanical ventilator support. A fulminant course of the disease, decreased values of complement levels and positive antinuclear antibodies (ANA) in pleural fluid and repeated negative sputum for acid-fast bacillus, blood cultures enabled diagnosis of fulminant lupus pneumonitis. Fulminant lupus pneumonitis is a rare but potentially life threatening complication of SLE. Management requires involvement of multiple specialties and rigorous efforts in reviving the patient.

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Treatment of Lupus Interstitial Lung Disease with Intravenous Cyclophosphamide

Cited by 60 publications

References 16 publications

Clinical Effects of Intermittent, Intravenous Cyclophosphamide in Severe Systemic Lupus erythematosus

Clinical Effects of Intermittent, Intravenous Cyclophosphamide in Severe Systemic Lupus erythematosus

Intermittent intravenous cyclophosphamide pulse therapy for the treatment of active interstitial lung disease associated with collagen vascular diseases

Systemic lupus erythematosus presenting as fulminant lupus pneumonitis: a rare case report

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