Subarachnoid hemorrhage in systemic lupus erythematosus in Japan: two case reports and a review of the literature

Abstract

We report 51- and 43-year-old Japanese female patients with systemic lupus erythematosus (SLE) associated with subarachnoid hemorrhage (SAH) due to rupture of intracranial saccular aneurysms. We also review the literature of Japanese SLE patients with SAH. SAH in Japanese SLE patients is more frequent than in patients from Western countries, has different features from the general population, and can occur regardless of SLE disease activity. Clinicians must pay attention to SAH in all SLE patients.     

Subarachnoid hemorrhage and systemic lupus erythematosus

The frequency, clinical profile, treatment and outcome of subarachnoid hemorrhage (SAH) in patients with systemic lupus erythematosus (SLE) were assessed retrospectively, based on the case records of SLE of the Jichi Medical School Hospital over a 20 year period. Clinically defined SAH was found in 10 (3.9%) out of 258 SLE patients, which represented a frequency higher than previously assumed. Five patients had active SLE and lacked an apparent cause of SAH, other than SLE. A high mortality rate (5/5), no visible aneurysm on angiogram (3/4), and an onset during intractable SLE or after discontinued or no steroid therapy because of medical noncompliance (4/5) were characteristic of patients with active SLE, and thus an earlier successful suppression of SLE, if possible, might have prevented their SAH. In contrast, in the 5 patients with inactive SLE, 2 out of 3 saccular aneurysms were successfully clipped and small bleeding of one patient without aneurysms remitted spontaneously without the need for additional steroid therapy. When one death, which occurred outside of medical care, was excluded, the survival ratio of the hospitalized SAH patients with inactive SLE was significantly better than that with active SLE (3/4 versus 0/5, P=0.0476). In conclusion, the relatively common occurrence of SAH in SLE patients, and a significantly different clinical impact of SAH in respect to active and inactive SLE, were suggested from the results.     

Optic neuropathy as a presenting feature of systemic lupus erythematosus: two case reports and literature review

Systemic lupus erythematosus (SLE) may affect the eyes and/or visual system in up to a third of patients; however, optic nerve involvement has been rarely reported. SLE presenting as optic neuropathy is exceptional. We report two new cases of optic neuropathy as a presenting feature of SLE. The first patient presented with an ischemic optic neuropathy and antiphospholipid antibodies, and the second presented with optic neuritis. A literature review for previous cases of SLE presenting as optic neuropathy was performed. Early diagnosis of SLE-associated optic neuropathy is warranted and leads to a better prognosis.     

Non‐aneurysmal subarachnoid hemorrhage in two patients with systemic lupus erythematosus: Case reports and literature review

We describe two cases of non‐aneurysmal subarachnoid hemorrhage (SAH) and multifocal stenosis of the intracranial arteries. The patients' histories together with magnetic resonance angiography, vessel wall imaging and transcranial Doppler (TCD) indicated that the SAH was due to vasculitis or reversible cerebral vasoconstriction syndrome (RCVS). Differential diagnosis of vasculitis and RCVS is important because the treatment strategies are different: immunosuppressants in vasculitis and calcium channel blockers in RCVS. Vessel wall magnetic resonance imaging and TCD can be helpful in differentiating them.     

Hemophagocytic syndrome as one of main manifestations in untreated systemic lupus erythematosus: two case reports and literature review

Hemophagocytic syndrome (HPS) is an unusual but fatal disorder characterized by pancytopenia and activation of macrophages. We describe two cases of untreated systemic lupus erythematosus (SLE) with HPS that presented as one of the manifestations of SLE. The onset of HPS was after parturition for one patient, and after abortion for the other. Bone marrow examination revealed severe hemophagocytosis in both patients. One patient responded to pulsed methylprednisone alone, and the other responded to pulsed methylprednisone plus intravenous immunoglobulin (IVIG). We believe accurate diagnosis, intensive therapy, and sufficient supportive cares are essential in improving patients’ prognosis.     

Association between myasthaenia gravis and systemic lupus erythematosus: three case reports and review of the literature

The coexistence of systemic lupus erythematosus (SLE) and myasthaenia gravis (MG) has been reported previously. Because of their shared clinical characteristics and autoantibody-mediated pathogenesis, an SLE expert panel decided to include MG as one of the 19 neuropsychiatric SLE syndromes. This study reports a cluster of three cases of SLE/MG overlap from our cohort and a review of the published data concerning this overlap of SLE and MG. A systematic Medline review revealed 13 cases described in eight publications from 1994 to 2009. In summary, 12 of the 16 patients (three from our cohort and 13 from the reported cases) were women with an average age of 34 years. The most common SLE manifestations were polyarthritis (15 out of 16 patients), skin rashes (5/16), serositis (5/16), and cytopaenias (10/16). All of the patients were anti-nuclear antibodies (ANA) positive and 15/16 were anti-dsDNA positive. Proximal muscle weakness was the most frequent MG-related symptom (9/16), while 11/16 patients were anti-acetylcholine receptor (anti-AChR) antibody positive and 9/16 had diagnostic electromyography (EMG). These data suggest that MG should to be included in the differential diagnosis of lupus patients with fatigue and muscular weakness together with inflammatory and drug-induced myopathy.     

Cardiac wall rupture in systemic lupus erythematosus: a case report and review of the literature

Clinical Rheumatology - Cardiac wall rupture (CWR) is a serious and often fatal complication of myocardial infarction (MI). Despite an increase in the incidence of MI in patients with systemic...     

系统性红斑狼疮合并膀胱炎及尿路梗阻一例并文献复习

目的提高对系统性红斑狼疮(SLE)合并膀胱炎及尿路梗阻的认识。方法报告1例特殊而临床少见的SLE合并膀胱炎、尿路梗阻及假性肠梗阻病例并复习相关文献。结果患者为37岁女性，SLE病史4年，出现膀胱刺激征1年，腹胀1个月；中段尿培养阴性；B超及膀胱镜示：膀朊壁增厚，膀胱容量减少，黏膜充血、水肿。X线及造影检查证实肠梗阻及尿路梗阻；激素及环磷酰胺治疗后膀胱炎及肠梗阻症状减轻。结论SLE合并的膀胱炎临床少见，属于间质性膀胱炎，是SLE合并尿路梗阻的最常见原因；SLE尿路梗阻常与假性肠梗阻同时出现，可能属于SLE的一个独特临床亚型；SLE合并肠梗阻病情严重，病死率高，临床上应予重视。     

Acquired hemophilia in a patient with systemic lupus erythematosus: a case report and literature review

We report the case of a 38-year-old female patient with systemic lupus erythematosus (SLE) who developed acquired hemophilia caused by factor VIII (FVIII) inhibitors. She manifested spontaneous bleeding symptoms such as ecchymoses and hematuria. Laboratory findings showed an isolated prolongation of the activated partial thromboplastin time, reduced FVIII activity, and a high titer of FVIII inhibitors. She was successfully treated with oral predonisolone and cyclosporine in combination with steroid and cyclophosphamide pulse therapy.     

Acquired hemophilia in a patient with systemic lupus erythematosus: a case report and literature review

Abstract

We report the case of a 38-year-old female patient with systemic lupus erythematosus (SLE) who developed acquired hemophilia caused by factor VIII (FVIII) inhibitors. She manifested spontaneous bleeding symptoms such as ecchymoses and hematuria. Laboratory findings showed an isolated prolongation of the activated partial thromboplastin time, reduced FVIII activity, and a high titer of FVIII inhibitors. She was successfully treated with oral predonisolone and cyclosporine in combination with steroid and cyclophosphamide pulse therapy.     

Late-onset systemic lupus erythematosus in Northwestern Spain: differences with early-onset systemic lupus erythematosus and literature review

To further investigate into the epidaemiology of systemic lupus erythematosus (SLE) in Southern Europe, we have assessed the incidence, clinical spectrum and survival of patients diagnosed with late-onset SLE (age?≥?50 years) according to the 1982 American College of Rheumatology (ACR) classification criteria at the single hospital for a well-defined population of Lugo, Northwestern (NW) Spain. Between January 1987 and December 2006, 51 (39.3%) of the 150 patients diagnosed as having SLE fulfilled definitions for late-onset SLE. The predominance of women among late-onset SLE (4:1) was reduced when compared with that observed in early-onset SLE (7:1). However, the incidence of late-onset SLE was significantly higher in women (4.2 [95% confidence interval (CI): 3.1-5.6] per 100,000 population) than in men (1.3 [95% CI: 0.6-2.2] per 100,000 population) (p?相似文献   

Silica-associated systemic lupus erythematosus with lupus nephritis and lupus pneumonitis: A case report and a systematic review of the literature

IntroductionSeveral epidemiological studies have shown that silica exposure triggers the onset of systemic lupus erythematosus (SLE); however, the clinical characteristics of silica-associated SLE have not been well studied.Patient concernsA 67-year-old man with silicosis visited a primary hospital because of a fever and cough. His respiratory condition worsened, regardless of antibiotic medication, and he was referred to our hospital.DiagnosisThe patient showed leukopenia, lymphopenia, serum creatinine elevation with proteinuria and hematuria, decreased serum C3 level, and was positive for anti-double stranded DNA antibody, anti-nuclear antibody, and direct Coombs test. He was diagnosed with SLE. Renal biopsy was performed, and the patient was diagnosed with lupus nephritis (class IV-G(A/C) + V defined by the International Society of Nephrology/Renal Pathology Society classification). Computed tomography revealed acute interstitial pneumonitis, bronchoalveolar lavage fluid showed elevation of the lymphocyte fraction, and he was diagnosed with lupus pneumonitis.InterventionsPrednisolone (50 mg/day) with intravenous cyclophosphamide (500 mg/body) were initiated.OutcomesThe patient showed a favorable response to these therapies. He was discharged from our hospital and received outpatient care with prednisolone slowly tapered off. He had cytomegalovirus and herpes zoster virus infections during treatment, which healed with antiviral therapy.Review:We searched for the literature on sSLE, and selected 11 case reports and 2 population-based studies. The prevalence of SLE manifestations in sSLE patients were comparative to that of general SLE, particularly that of elderly-onset SLE. Our renal biopsy report and previous reports indicate that lupus nephritis of sSLE patients show as various histological patterns as those of general SLE patients. Among the twenty sSLE patients reported in the case articles, three patients developed lupus pneumonitis and two of them died of it. Moreover, two patients died of bacterial pneumonia, one developed aspergillus abscesses, one got pulmonary tuberculosis, and one developed lung cancer.ConclusionClose attention is needed, particularly for respiratory system events and infectious diseases, when treating patients with silica-associated SLE using immunosuppressive therapies.     

Celiac disease in a patient with systemic lupus erythematosus: a case report and review of literature

Celiac disease (CD) is an inflammatory condition of the gut with a known autoimmune pathogenesis. Many similarities exist between the pathogenesis of CD and systemic lupus erythematosus (SLE); it is still unknown whether there is an association. There are 13 case reports in the literature of both diseases occurring simultaneously. We report another patient who was diagnosed with SLE and 8 years later, developed CD. A review of the literature is also presented.     

Refractory hemophagocytic syndrome in systemic lupus erythematosus successfully treated with intermittent intravenous cyclophosphamide: three case reports and literature review

We experienced three patients with refractory or severe hemophagocytic syndrome associated with the activity of systemic lupus erythematosus, so-called acute lupus hemophagocytic syndrome (ALHS). All patients were successfully treated with intermittent intravenous injections of cyclophosphamide (IVCY). In each patient, hemophagocytosis was found during bone marrow examination, and infectious causes of hemophagocytic syndrome were carefully excluded. Patients 1 and 2 were refractory to combination therapy with a high-dose corticosteroid and rituximab or cyclosporine and were successfully treated with additive IVCY. Patient 3 had very severe ALHS that developed after cardiac surgery but was successfully treated with a high-dose corticosteroid and IVCY. We conducted a literature review of adult ALHS and analyzed 54 cases reported from 2006 to 2013. Although the clinical and laboratory features were diverse, fever, bicytopenia or pancytopenia, and hyperferritinemia were almost universal. Including our 3 patients, a total of 16 have reportedly been treated with IVCY-containing regimens, and ALHS was successfully controlled in most of them without switching or adding other therapies. We suggest that combination therapy with IVCY and corticosteroids may be useful to treat severe or refractory ALHS.     

Acute intermittent porphyria and systemic lupus erythematosus: report of a case and review of the literature

The case of a Greek woman with acute intermittent porphyria (AIP) associated with systemic lupus erythematosus (SLE) is described and the literature on this association is reviewed. The coexistence of these two diseases may be determined by unknown mechanisms or could be fortuitous.