Infectious complications in SLE after immunosuppressive therapies

Immunosuppressive drugs have become the gold standard for the treatment of major organ involvement in systemic lupus erythematosus. The use of immunosuppressive therapy in systemic lupus erythematosus carries significant risks for infection. This article reviews infectious complications in systemic lupus erythematosus, focusing on effects of immunosuppressive therapy. Patients with systemic lupus erythematosus appear to carry an intrinsically increased risk for infection. Recent studies support this notion further by showing increased risk for serious infections in patients with systemic lupus erythematosus who had mannose-binding lectin deficiency associated with homozygous mannose-binding lectin variant alleles. Patients with systemic lupus erythematosus who were homozygous for mannose-binding lectin variant alleles had a fourfold increase in the incidence of infections, requiring hospitalization. In terms of extrinsic risk factors for infection, use of steroids and cyclophosphamide are the strongest risk factors. The effect of these drugs on infection is also dose dependent. The incidence of infectious complications in patients treated with mycophenolate mofetil, a newly used immunosuppressive drug in systemic lupus erythematosus, appears less frequent compared with cyclophosphamide. Herpes zoster is still the most common viral infection in patients with systemic lupus erythematosus treated with cyclophosphamide and mycophenolate mofetil. Overall data indicate that patients with systemic lupus erythematosus may have intrinsically increased risks for infection that are augmented by immunosuppressive therapies. Cyclophosphamide, in particular in combination with high-dose glucocorticoids, has the strongest effect in suppressing the immune responses against microorganisms. Careful monitoring of infectious complications is warranted in patients with systemic lupus erythematosus receiving immunosuppressive therapies, in particular those on high-dose glucocorticoids and cytotoxic drugs.     

MORTALITY OF JAMAICAN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS

A retrospective study of all patients with systemic lupus erythematosus(SLE) who died at the University Hospital of the West Indiesover a 14-year period is presented. The major cause of deathwas infection followed by renal failure. Gram-negative organismswere the major microbiological agents causing infections. Side-effectsof therapy were common, in particular bone marrow depressionand haemorrhage related to anticoagulants. It appears that controllingsevere lupus activity without increasing the risk of life-threateningcomplications remains an important goal in the treatment ofSLE. KEY WORDS: Systemic lupus erythematosus, Mortality, Infection, Anticoagulants, Jamaica     

Opportunistic infections mimicking gastrointestinal vasculitis in systemic lupus erythematosus.

Patients with systemic lupus erythematosus who are on chronic immunosuppressive therapy are at risk for developing infectious complications. We present 2 cases of immunosuppressed patients with systemic lupus erythematosus who presented with abdominal complaints without other systemic lupus symptoms. These patients were initially thought to have gastrointestinal vasculitis based on preliminary pathologic reports; however, further workup and careful review of the pathologic specimens confirmed an opportunistic infection as the etiology in each case. It is critical that physicians maintain a high index of suspicion for infection when treating immunocompromised patients with systemic lupus erythematosus with abdominal complaints to avoid delay in appropriate treatment.     

Disseminated Zygomycosis Simulating Cerebrovascular Disease and Pulmonary Alveolar Haemorrhage in a Patient with Systemic Lupus Erythematosus

Patients with systemic lupus erythematosus (SLE) may be complicated with serious cerebrovascular accidents and pulmonary alveolar haemorrhage. The authors report an autopsy-proven angioinvasive zygomycosis in a patient with SLE. The clinical features of systemic zygomycosis in this patient masqueraded as SLE-related stroke and pulmonary haemorrhage. The case demonstrates that the simultaneous occurrence of complications that clinically suggest pulmonary haemorrhage and multiple brain infarcts in SLE patients should include the rare disseminated zygomycosis in the differential diagnosis. Received: 17 March 1999 / Accepted: 20 September 1999     

Nocardia infection of a joint prosthesis complicating systemic lupus erythematosus

The authors report the case of a 43-year-old woman suffering from severe systemic lupus erythematosus treated with long-term prednisone, who developed Nocardia nova infection on a hip prosthesis. Sepsis occurred about two years after an episode of pulmonary nocardiosis with the same Nocardia species, that was successfully treated by 12 months of antibiotics. A good outcome of the joint infection was observed in response to antibiotics and removal of the prosthesis. Nocardiosis is a rare infection, acting as an opportunistic infection, facilitated in the present case by systemic lupus erythematosus and chronic corticosteroid therapy. Nocardia infections mainly affect the lungs, skin and central nervous system; these last two sites are mostly due to haematogenous spread, a frequent event. Treatment is based on antibiotics, usually continued for 3-12 months, especially because of the risk of relapse. The imipenem-amikacin combination appears to be more effective than trimethoprim sulfamethoxazole. To our knowledge, this is the first case report of Nocardia nova joint prosthesis infection also presenting as late septic spread of pulmonary nocardiosis, complicating corticosteroid-treated systemic lupus erythematosus.     

Transverse myelopathy in systemic lupus erythematosus. Report of three cases and review of the literature.

Three cases of transverse myelopathy associated with systemic lupus erythematosus were reported, and 23 similar cases previously reported were reviewed. A diagnosis of systemic lupus erythematosus was made in only 60% before the onset of transverse myelopathy. The time of onset of myelopathy was randomly distributed during the disease. The most common presenting neurologic symptom was numbness, or weakness of the legs, or both. A unique association between the acute stage of transverse myelopathy and marked reduction of cerebrospinal fluid glucose concentration was observed. Thirteen patients died, 9 had permanent neurologic deficits, and only 4 recovered nearly normal function. Eight patients had ischemic necrosis of the spinal cord at postmortem examination, and vascular lesions were found in the spinal cord of 3 additional patients. The value of steroid treatment was uncertain. Patients who were started on steroid therapy within 24 hours of the onset of myelopathy may have benefited.     

Pulmonary haemorrhage and mesenteric vasculitis in a patient with systemic lupus erythematosus

WE Report a 26‐year‐old female patient with systemic lupus erythematosus (SLE) who developed mesenteric vasculitis and pulmonary haemorrhage. This patient initially presented with an acute abdomen and extensive vasculitic rash. While she was being treated for her abdomen she developed fulminant pulmonary haemorrhage requiring mechanical ventilation. With supportive measures and aggressive immunosupression treatment she eventually made a complete recovery.     

Pulmonary haemorrhage, pulmonary infarction, and the lupus anticoagulant.

A patient with systemic lupus erythematosus developed pulmonary haemorrhage and pulmonary infarction as rare initial manifestations of her disease. The latter was associated with the presence of the circulating lupus anticoagulant. She recovered with pulse doses of methylprednisolone and plasmapheresis. Anticoagulants were not administered.     

Pyrexia and normal C-reactive protein (CRP) in patients with systemic lupus erythematosus: always consider the possibility of infection in febrile patients with systemic lupus erythematosus regardless of CRP levels

SIR, Patients with active systemic lupus erythematosus (SLE)often have normal C-reactive protein (CRP) levels. However,bacterial infections can elicit CRP production in these patients[1, 2]. It has therefore been proposed that measuring the CRPlevels in pyrexial patients with SLE can help to differentiatebetween active disease and infection [1, 2]. We describe twopatients with SLE with systemic bacterial infection who presentedwith normal CRP levels. The first patient was     

来氟米特联合糖皮质激素治疗增殖型狼疮性肾炎的多中心对照临床试验研究

目的观察来氟米特治疗增殖型狼疮性肾炎(LN)的疗效及安全性。方法多中心对照临床试验。选病理证实的活动性增殖型LN患者,对既往从未接受过免疫抑制剂治疗者,在应用激素的基础上分别口服来氟米特(A组)或静点环磷酰胺(B组);对3个月前接受过免疫抑制剂和激素治疗后复发者也给予来氟米特(C组)治疗。用药期间监测血压、血尿常规、24h尿蛋白定量、肝肾功能、抗核抗体及抗双链DNA抗体滴度、血沉和补体C3等。在第3、6个月时行狼疮疾病活动指数(SLEDAI)评价。记录所有不良反应。6个月后行疗效和安全性的评价。结果共有51例患者参加了本试验,A组20例,B组16例,C组15例;有4例患者因不良反应退出试验,47例患者如期完成了试验。A组来氟米特治疗总有效率达80%,完全缓解率为40%;B组环磷酰胺治疗总有效率达75%,完全缓解率为25%;组间比较,差异无统计学意义。治疗6个月后,A、B组患者各项指标(尿蛋白、血清白蛋白、血肌酐、SLEDAI)均有明显改善,但组间比较差异无统计学意义。C组来氟米特治疗总有效率达60%,完全缓解率为6·7%。A组不良反应主要是感染和脱发,感染以带状疱疹多见,有1例患者发生严重肺部感染。结论来氟米特联合激素用于活动性增殖型LN的诱导缓解治疗有较明显的疗效,耐受性尚好。其在维持缓解期的长期疗效及安全性有待更长期的观察。     

Presentation and prognosis of shrinking lung syndrome in systemic lupus erythematosus: report of four cases

Systemic lupus erythematosus is an autoimmune systemic disease that commonly affects the respiratory system. Shrinking lung syndrome is a rare respiratory complication associated with systemic lupus erythematosus. Patients present with dyspnea alone or associated with chest pain and orthopnea, lung volume reduction with no parenchymal abnormalities and a restrictive ventilatory defect on pulmonary function tests. The pathogenesis, treatment, and prognosis of shrinking lung syndrome remain controversial. This study describes the clinical features, investigations, and outcome of a series of four patients with systemic lupus erythematosus and shrinking lung syndrome regularly followed on Rheumatology Service of the Clinics Hospital of the Federal University of Minas Gerais, Brazil, with a brief review of literature. It emphasizes that, despite prognosis of shrinking lung syndrome has been reported as good, it may cause severe functional pulmonary abnormalities and must be treated promptly and aggressively in order to, at least, stabilize pulmonary function tests.     

Systemic lupus erythematosus presenting with features suggestive of human immunodeficiency virus infection

We describe 8 patients who presented with fever, weight loss, anemia, and oral and/or esophageal candidiasis, and who were initially thought to have human immunodeficiency virus (HIV) infection or lymphoma. These patients fulfilled American College of Rheumatology criteria for systemic lupus erythematosus (SLE) because of arthralgias or arthritis, hematological derangements, and immunological abnormalities. Treatment was delayed because SLE did not immediately enter into the differential diagnosis. All patients had a rapid response to corticosteroids, with defervescence of fever, decrease in lymphadenopathy within 24-48 hours, and complete resolution of lymphadenopathy and other signs and symptoms of illness in 7-10 days. It is important to recognize this mode of SLE presentation in patients who test negative for HIV infection so that the appropriate diagnostic evaluation and initiation of treatment can be expedited.     

A case of acute pericarditis with hemophagocytic syndrome,cytomegalovirus infection and systemic lupus erythematosus

Hemophagocytic syndrome, cytomegalovirus infection and systemic lupus erythematosus (SLE) would each be critical diseases separately. Viral infections, autoimmune diseases or malignancies can complicate the Hemophagocytic syndrome. Cytomegalovirus infection is known to be prevalent in immune compromised hosts, and can exacerbate the symptoms of systemic lupus erythematosus. A 25-year-old man presented with fever and acute pericarditis with the hemophagocytic syndrome, cytomegalovirus infection and systemic lupus erythematosus; all developed concurrently at the onset of illness. With treatment, using ganciclovir and glucocorticoid medication the patient improved. Here we report this rare case and review the medical literature.     

Invasive pulmonary aspergillosis and pulmonary cryptococcosis really coexist in immunocompromised host

Fungal infections develop slowly in immunocompetent patients and rarely a severe disease, however, they progress rapidly and usually prove fatal in immunocompromised patients. Early diagnosis and treatment of fungal infections is essential to survival of immunocompromised patients. We report a 33-year-old woman with systemic lupus erythematosus undergoing immunotherapy infected with combined invasive pulmonary aspergillosis and pulmonary cryptococcosis diagnosed by video-assisted thoracic surgery lung biopsy and she was successfully treated as a result of early diagnosis and treatment.     

Detection of anti-dsDNA as a diagnostic tool: a prospective study in 441 non-systemic lupus erythematosus patients with anti-dsDNA antibody (anti-dsDNA).

The diagnostic significance of anti-double-stranded deoxyribonucleic acid (anti-dsDNA) determination was evaluated in a prospective manner from 1974 to 1982 in a group of 441 patients without systemic lupus erythematosus whose sera were found to contain antibodies to dsDNA on routine screening (Farr assay). Within one year 69% (304) of these patients fulfilled the preliminary American Rheumatism Association (ARA) criteria for systemic lupus erythematosus (SLE). Eighty-two of the remaining 137 patients were followed up for several years. At the end of the study 52% of these patients had also developed systemic lupus erythematosus. Patients who developed systemic lupus erythematosus were characterised by the occurrence of relatively high avidity anti-dsDNA in the circulation compared with patients who did not develop systemic lupus erythematosus. It can be concluded that about 85% of patients without systemic lupus erythematosus with anti-dsDNA in the circulation will develop SLE within a few years. Taking into account the relative avidity of anti-dsDNA, as determined by calculation of Farr/polyethylene glycol (PEG) ratios, we conclude that patients with relatively high avidity anti-dsDNA are more prone to develop systemic lupus erythematosus than patients with relatively low avidity anti-dsDNA.     

Systemic lupus erythematosus and vaccination

Patients with systemic lupus erythematosus (SLE) are frequently immunodepressed making them more vulnerable to infections. Preventive vaccination is therefore warranted but has often been withheld owing to fears of a link between infection and autoimmunity, and the possibility of inducing or exacerbating lupus after vaccination. The data published in the literature suggest that vaccination of lupus patient is safe, except for live vaccines. Their efficacy is lower than in healthy subjects but protection seems to be sufficient. But further large-scale studies are required to confirm these statements.     

How to manage patients with cardiopulmonary disease?

Systemic lupus erythematosus (SLE) is a connective tissue disease characterized by the formation of autoantibodies and immune complexes. The heart and lungs are among the organ systems commonly affected in SLE. Pericarditis, premature coronary atherosclerosis, pleuritis and pulmonary infections are the most prevalent cardiopulmonary manifestations. Other rare associations include myocarditis, coronary arteritis, acute lupus pneumonitis/pulmonary haemorrhage, acute reversible hypoxaemia and 'shrinking lung' syndrome. Current imaging modalities may provide earlier detection of subclinical disease, which may aid in preventing these potentially fatal complications. The response to treatment varies, depending on the presentation of disease. In this chapter we address the frequency, diagnosis and monitoring, and treatment regimens of cardiac and pulmonary involvement in patients with SLE.     

Polyarticular Salmonella bacterial arthritis in a patient with systemic lupus erythematosus

Patients with systemic lupus erythematosus (SLE) are at increased risk for infections. Insufficiency of the reticuloendothelial system caused either by immunosuppressive therapy or inadequate opsonisation are mechanisms by which Salmonella infections in particular appear in these patients. Salmonella infections can provoke a polyarticular reactive arthritis while Salmonella bacterial arthritis usually is monarticular. We report on the seriousness of a Salmonella infection (enteritis complicated by polyarticular bacterial arthritis) in a patient with SLE.     

系统性红斑狼疮并发感染的危险因素临床特点及早期诊断

感染是影响系统性红斑狼疮(SLE)患者病死率的重要因素之一,故早期诊断感染并发症能显著改善SLE患者预后。血清C-反应蛋白(CRP)、降钙素原(PCT)有助于鉴别SLE并发感染和SLE活动,血清KL-6有助于鉴别SLE伴发感染和SLE累及肺脏。SLE合并感染的病原体除了最多见的细菌感染外,病毒和真菌感染近几年有上升趋势,需要引起足够重视。