系统性红斑狼疮伴颅内环形强化病灶

报道1例重症系统性红斑狼疮患者,病程中发生严重的血小板减少伴支气管出血,急性胰腺炎,同时头颅MRI检查发现颅内环形强化病灶,肾活检病理诊断为狼疮性肾炎Ⅳ型伴血栓性微血管病。经积极治疗,狼疮活动性病变控制,各器官功能改善,但颅内病灶增大,经脑活检确诊为颅内曲霉菌感染,予手术切除病灶及抗曲霉菌治疗,预后良好。     

Central nervous system infections in Filipino patients with systemic lupus erythematosus

Background and purpose: Infections including those of the central nervous system (CNS) are a major contributor to morbidity and mortality in systemic lupus erythematosus (SLE). This case series describes the etiology, contributing factors and outcomes of CNS infections in a group of Filipino patients with SLE. Design: Retrospective case series. Methods: We reviewed the medical records of SLE patients diagnosed and confined for a CNS infection at the University of Santo Tomas Hospital in Manila, Philippines, from 1997 to 2007. Results: A total of 23 SLE patients (22 females) diagnosed with CNS infection were included in this study. The mean age was 25.8 years (range 12–51) at SLE diagnosis, and 30.9 years (range 14–58) at CNS infection, with a mean disease duration of 55 months (range 7–125). Nineteen cases (82.6%) were meningitis, and four (17.4%) were diagnoses of brain abscess. The etiologic agents were identified as Cryptococcus neoformans in seven (30.4%), Mycobacterium tuberculosis in seven (30.4%), Streptococcus pneumoniae in two (8.7%), Salmonella sp. in one (4.4%), Corynebacterium bovis with Actinomyces sp. in one (4.4%), and no isolate in five (21.7%). The average daily prednisone dose was 28.9 mg (range 0–60 mg); 10 patients had recently received pulse cyclophosphamide, and two were on mycophenolate mofetil at the time of infection. Most cases had active SLE; the lone patient in disease remission had S. pneumoniae meningitis post‐splenectomy. The most common presentation was headache (100%) and fever (87%). The infection resolved completely in nine patients (39.1%), and resolved with sequelae in two patients (8.7%); 12 patients (52.2%) died. Conclusion: We described the etiology and outcomes of CNS infections in a group of Filipino patients with SLE. Risk factors included active SLE in the majority of cases requiring moderate‐ to high‐dose steroids and other immunosuppressants like cyclophosphamide. Although C. neoformans and M. tuberculosis were the most common etiologic agents, it is just as important to search for less common organisms which can produce disease in highly susceptible hosts. A high index of suspicion and early appropriate management are crucial to favorable outcome among these patients.     

Treatment of acute neuropsychiatric lupus with intravenous immunoglobulin (IVIG): a case report and review of the literature

Neuropsychiatric lupus can be difficult to diagnose, and little prospective data exists to help direct management. In this case report we describe the acute onset of symptoms of depression, mania, and psychosis and their complete resolution 48 h following a 5-day treatment course of intravenous immunoglobulin (IVIG) in a 20-year-old woman with systemic lupus erythematosus (SLE). We review the literature on IVIG for the management of neuropsychiatric lupus. We propose that when more toxic therapies are refused or symptoms do not remit with other treatments, IVIG should be considered in patients with neuropsychiatric lupus.     

A case of systemic lupus erythematosus associated with trigger wrist

A 54-year-old woman complained of a painful, swollen, and clicking left wrist for 1 year. She had an 8-year history of systemic lupus erythematosus (SLE), treated with oral prednisolone. Flexion and extension of all fingers were difficult to initiate on wrist extension, and movement was accompanied by a palpable click at the wrist. Magnetic resonance imaging (MRI) and tenography revealed the expanded sheath of the flexor tendons and well-defined round free bodies known as rice bodies. Synovectomy and excision of rice bodies resulted in complete disappearance of swelling and triggering of the wrist. Received: August 23, 2000 / Accepted: July 6, 2001     

Systemic lupus erythematosus in identical twins: a case report

In this report we describe the case of identical twin sisters that developed systemic lupus erythematosus (SLE). These patients have in common major histocompatibility complex class I and class II alleles and identical red blood cell antigens, which is a clear indication of monozygotic twins. Both twins showed high titers of anti-dsDNA antibody. However, only one of them manifested signs of lupus psychosis and was positive for the LE test, rheumatoid factor, anti-Scl 70, anti-SSA, and antiribosomal P antibodies. Both sisters lived together; therefore, the environmental factors were considered to be the same. Interestingly, these patients expressed different types of autoantibodies and the manifestation of disease was also quite different. When one of the twins was diagnosed with SLE, we began to closely follow up signs of the disease in the other twin periodically. This enabled us to promptly diagnose the second twin with SLE and she was successfully treated without progression of the disease. It is important to mention that following up the subsequent history of an identical twin diagnosed with SLE allowed early detection of the disease in the other twin.     

Acute pan-dysautonomia as well as central nervous system involvement and peripheral neuropathies in a patient with systemic lupus erythematosus

Abstract

A 32-year-old woman was diagnosed with leucopenia in 2002, being antinuclear antibody, anti-DNA antibody, and antiphospholipid antibody positive, and she was administered low-dose aspirin. In July 2006, she was admitted to our hospital because of pyrexia and abdominal pain. Examination revealed paralytic ileus, absence of the pupillary light reflex, dyshidrosis and anuresis. In addition, with high-level interleukin-6 in cerebrospinal fluid, the sensory nerve conduction velocity was derivation impotence. She was subsequently diagnosed with systemic lupus erythematosus (SLE) with central nervous system involvement, peripheral neuropathy as well as acute pan-dysautonomia. After pulse corticosteroid therapy, paralytic ileus was improved, however, the urination disorder persisted, and syncope due to orthostatic hypotension became marked. Plasma exchange and a second course of pulse corticosteroid therapy were performed, and were ineffective, whereas intravenous cyclophosphamide was effective. This patient is a rare case of central nervous system, peripheral neuropathy as well as acute pan-dysautonomia with SLE.     

Acute pan-dysautonomia as well as central nervous system involvement and peripheral neuropathies in a patient with systemic lupus erythematosus

A 32-year-old woman was diagnosed with leucopenia in 2002, being antinuclear antibody, anti-DNA antibody, and antiphospholipid antibody positive, and she was administered low-dose aspirin. In July 2006, she was admitted to our hospital because of pyrexia and abdominal pain. Examination revealed paralytic ileus, absence of the pupillary light reflex, dyshidrosis and anuresis. In addition, with high-level interleukin-6 in cerebrospinal fluid, the sensory nerve conduction velocity was derivation impotence. She was subsequently diagnosed with systemic lupus erythematosus (SLE) with central nervous system involvement, peripheral neuropathy as well as acute pan-dysautonomia. After pulse corticosteroid therapy, paralytic ileus was improved, however, the urination disorder persisted, and syncope due to orthostatic hypotension became marked. Plasma exchange and a second course of pulse corticosteroid therapy were performed, and were ineffective, whereas intravenous cyclophosphamide was effective. This patient is a rare case of central nervous system, peripheral neuropathy as well as acute pan-dysautonomia with SLE.     

A case of very-late-onset systemic lupus erythematosus

Abstract

A 93-year-old woman was admitted to our hospital because of fever. Radiographic findings revealed accumulation of pleural fluid. Moreover, blood tests revealed inflammation, lymphopenia, hypocomplementemia, positive for anti-nuclear antibody, and elevated anti-DNA antibody level. Therefore, the patient was diagnosed with pleuritis associated with systemic lupus erythematosus (SLE). Administration of prednisolone 20?mg/day resulted in a marked improvement in fever, pleuritis, and laboratory findings. We report a case of very-late-onset SLE that occurred at the age of 93.     

Autoimmune hepatitis in a patient with systemic lupus erythematosus: a case report

A 48-year-old woman was admitted to our hospital because of ascites. Laboratory data indicated the presence of systemic lupus erythematosus (SLE) with nephrotic syndrome and elevated hepatic enzymes. Treatment with prednisolone resulted in a marked clinical improvement in renal and liver dysfunction. Histopathologic analysis of renal and liver tissues showed lupus nephritis and liver cirrhosis, respectively. According to the autoimmune hepatitis scoring system, the patient had both SLE and autoimmune hepatitis.     

Autoimmune hepatitis in a patient with systemic lupus erythematosus: a case report

Abstract

A 48-year-old woman was admitted to our hospital because of ascites. Laboratory data indicated the presence of systemic lupus erythematosus (SLE) with nephrotic syndrome and elevated hepatic enzymes. Treatment with prednisolone resulted in a marked clinical improvement in renal and liver dysfunction. Histopathologic analysis of renal and liver tissues showed lupus nephritis and liver cirrhosis, respectively. According to the autoimmune hepatitis scoring system, the patient had both SLE and autoimmune hepatitis.     

A case of very-late-onset systemic lupus erythematosus

A 93-year-old woman was admitted to our hospital because of fever. Radiographic findings revealed accumulation of pleural fluid. Moreover, blood tests revealed inflammation, lymphopenia, hypocomplementemia, positive for anti-nuclear antibody, and elevated anti-DNA antibody level. Therefore, the patient was diagnosed with pleuritis associated with systemic lupus erythematosus (SLE). Administration of prednisolone 20 mg/day resulted in a marked improvement in fever, pleuritis, and laboratory findings. We report a case of very-late-onset SLE that occurred at the age of 93.     

Rapidly progressing central nervous system involvement despite of steroid pulse therapy in a case of systemic lupus erythematosus presented as mononeuritis multiplex

A 31‐year‐old‐woman with a 6‐month history of systemic lupus erythematosus (SLE) was admitted because of severe mononeuritis multiplex. Although she initially responded well with steroid pulse therapy, she developed unconsciousness again 3 weeks later just after a second course of steroid pulse. Laboratory evaluations revealed a high titre of anti‐DNA antibody, low complement levels, and positive lupus anticoagulant. She was treated with intravenous cyclophosphamide pulse therapy with immediate response. The clinical course of central nervous system involvement was very unusual for the response to steroid pulse therapy.     

Auditory disorders in patients with systemic lupus erythematosus: Relation to clinical parameters

Aim of the workTo evaluate the hearing disorders in SLE patients with particular regard to their frequency and relationship to disease duration and activity.Patients and methodsTwenty female SLE patients were enrolled in the study. Assessment of disease activity was done using the SLE disease activity index (SLEDAI). Another 20 otologically healthy subjects of matched age and sex served as controls. Auditory assessment was performed and included otoscopic examination, pure tone audiometry (PTA), acoustic immittance testing and speech audiometry.ResultsThe PTA was abnormal in 13 (65%) patients; 4 had tinnitus and 1 vertigo. The PTA results showed a highly significant statistical difference from the control (p < 0.001). Otoscopic examination, acoustic immittance testing and speech audiometry of all patients were normal. A significant difference was found in the age at disease onset between those with and without abnormal PTA (p = 0.023). Moreover, there was a significantly lower hearing level (right ear) at 12,000 Hz in juvenile-onset (N = 6) (20.83 ± 3.76 db) compared to adult-onset cases (32.5 ± 15.66 db) (p = 0.02). No significant difference was present in the audiovestibular manifestations (p = 0.114), clinical, laboratory parameters or disease activity between those with or without hearing loss. However, hearing levels were significantly lower in those with lupus nephritis and those receiving hydroxychloroquine.ConclusionPure tone audiometry revealed SNHL in 65% of SLE patients. Absence of audiovestibular manifestations does not exclude inner ear affection. Age at disease onset is remarkably associated with hearing loss in SLE. Lupus nephritis and hydroxychloroquine use are associated with lower hearing levels and possible early hearing loss.     

Left vocal cord paralysis in systemic lupus erythematosus

Abstract

Left vocal cord palsy is a well-recognised rare complication of SLE. We encountered a gentleman who during an acute flare developed left vocal fold palsy (VCP). Immunosuppression was increased, but this only produced partial improvement. VCP is commonly but not exclusively caused by recurrent laryngeal nerve palsy. Other causes may affect phonation, including mucosal ulceration, vasculitis and pulmonary hypertension. There is limited literature on the diagnosis of and treatment options for VCP in SLE.     

Left vocal cord paralysis in systemic lupus erythematosus

Left vocal cord palsy is a well-recognised rare complication of SLE. We encountered a gentleman who during an acute flare developed left vocal fold palsy (VCP). Immunosuppression was increased, but this only produced partial improvement. VCP is commonly but not exclusively caused by recurrent laryngeal nerve palsy. Other causes may affect phonation, including mucosal ulceration, vasculitis and pulmonary hypertension. There is limited literature on the diagnosis of and treatment options for VCP in SLE.     

Clinical research in systemic lupus erythematosus: immediate relevance to clinical practice

Systemic lupus erythematosus remains a challenge because of its diverse presentations, variable natural history, and lack of uniform response to treatment. True remission is very rare. Reliance on corticosteroid treatment leads to unwanted long-term toxicity. Great advances have been made in the early detection of lupus nephritis and in treatment. Greater appreciation of cognitive impairment and of lupus myelitis is now possible. Pregnancy risks are better characterized. However, the greatest unmet challenge remains atherosclerosis.     

Thrombotic thrombocytopenic purpura as an etiology of thrombocytopenia in systemic lupus erythematosus: case report

Abstract

Thrombotic thrombocytopenic purpura (TTP) is an unusual complication of systemic lupus erythematosus (SLE). Although the reported association between SLE and TTP is increasing, a few cases do improve without plasmatherapy. We report a case of TTP which was successfully treated without plasmatherapy, which might be underestimated as an etiology of thrombocytopenia in SLE. TTP should always be considered as a concomitant disease when Coombs' negative hemolytic anemia or thrombocytopenia is seen in SLE patients.     

Thrombotic thrombocytopenic purpura as an etiology of thrombocytopenia in systemic lupus erythematosus: case report

Thrombotic thrombocytopenic purpura (TTP) is an unusual complication of systemic lupus erythematosus (SLE). Although the reported association between SLE and TTP is increasing, a few cases do improve without plasmatherapy. We report a case of TTP which was successfully treated without plasmatherapy, which might be underestimated as an etiology of thrombocytopenia in SLE. TTP should always be considered as a concomitant disease when Coombs' negative hemolytic anemia or thrombocytopenia is seen in SLE patients.     

Catastrophic transverse myelitis in a patient with systemic lupus erythematosus

A 24-year-old Japanese woman was admitted to our hospital suffering from high fever and progressive paralysis in both legs. Magnetic resonance imaging of the spinal cord showed high-intensity signals from C5 to Th4 and from Th7 to L1 on T2-weighted images. The patient was diagnosed as having acute transverse myelitis , which was a complication of systemic lupus erythematosus based on the serological findings. Despite aggressive immunosuppressive treatments including corticosteroid pulse therapy, plasmapheresis, and intravenous cyclophosphamide, the paralysis of her lower extremities did not improve. In the catastrophic type of lupus-associated TM, which develops extensively and longitudinally along the spinal cord, the prognosis still seems to be poor despite intensive treatments.