Successful rituximab therapy in a lupus patient with diffuse alveolar haemorrhage

Diffuse alveolar haemorrhage (DAH) is a rare but life-threatening complication of systemic lupus erythematosus (SLE). Specific therapy is based on a heavy immunosuppressive treatment that usually associates corticosteroid and cyclophosphamide boluses and plasma exchange. Despite this treatment, an early mortality rate of 20-50% is reported in the literature. Immunosuppression-related complications are responsible for further mortality and morbidity. Rituximab, a specific anti-CD20 antigen B-cell antibody, has been used with success for the treatment of several refractory autoimmune disorders, but rarely for SLE-induced DAH. We report here the first case of SLE-induced DAH treated successfully with rituximab without cyclophosphamide administration in a patient intolerant to cyclophosphamide. We review the two other cases of SLE-induced DAH managed with rituximab as a part of the immunosuppressive regimen.     

Predictors of mortality in diffuse alveolar haemorrhage associated with systemic lupus erythematosus

The objective of this study was the evaluation of clinical, demographic and treatment-associated mortality factors in patients with diffuse alveolar haemorrhage (DAH) associated with systemic lupus erythematosus (SLE). Clinical, laboratory test, SLEDAI-2K, predictors of mortality (APACHE II) and different treatments including cyclophosphamide, methylprednisolone and rituximab were evaluated in SLE patients who were diagnosed with DAH, to determine potential association with mortality. Twenty-nine episodes of DAH in 22 SLE patients were included (one patient with four episodes, four patients with two episodes (seven recurrences)), 15 died. Mean age was 25.1 years and 1.5 years of SLE evolution with haemoglobin drop 3.4?g/dl. In 4 of 22 patients, the DAH diagnosis was confirmed by autopsy. Six episodes were in patients under 18 years of age (2 patients with recurrence). DAH was the initial manifestation of SLE in 10 patients. Of the 22 patients, 17 were women and 22/29 had DAH episodes. Dyspnoea and nephritis occurred in all patients, less common were arthritis (75.9%) and fever (65.5%); haemoptysis was present only in 44.8%. Through evaluation of all included factors, only thrombocytopenia, renal failure, requirement for mechanical ventilation and high APACHE II were associated with higher mortality. Cyclophosphamide use was associated with less mortality (not statistically significant).     

Recurrent thrombotic thrombocytopenic purpura in a patient with systemic lupus erythematosus

We encountered a 39-year-old female patient with systemic lupus erythematosus (SLE) in whom thrombotic thrombocytopenic purpura (TTP) recurred. The patient was successfully treated with corticosteroid in combination with immunosuppressive agents. Because TTP complicating SLE is more resistant to treatment than idiopathic TTP, prompt diagnosis and efficacious initial treatment are critical.     

Recurrent thrombotic thrombocytopenic purpura in a patient with systemic lupus erythematosus

Abstract

We encountered a 39-year-old female patient with systemic lupus erythematosus (SLE) in whom thrombotic thrombocytopenic purpura (TTP) recurred. The patient was successfully treated with corticosteroid in combination with immunosuppressive agents. Because TTP complicating SLE is more resistant to treatment than idiopathic TTP, prompt diagnosis and efficacious initial treatment are critical.     

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.     

Progressive multifocal leucoencephalopathy in a patient with systemic lupus erythematosus treated with rituximab

SIR, Progressive multifocal leucoencephalopathy (PML) is a rarebut usually fatal demyelinating disease of the brain causedby JC papovavirus (JCV). At least 50–75% of the adultpopulation are seropositive for JCV. When JCV reactivation occurs,focal plaques develop in central nervous system white matter.Viral proliferation causes lysis of oligodendrocytes and thereforerapid demyelination [1]. Rituximab is a relatively novel therapyfor systemic lupus erythematosus (SLE) and PML has not previouslybeen reported in a patient with SLE treated with rituximab. The patient was     

Successful long-term treatment of systemic lupus erythematosus with rituximab maintenance therapy

Systemic lupus erythematosus (SLE) is a chronic, inflammatory autoimmune disease that may involve multiple organ systems. Treatment consists of immunosuppression, cytotoxic treatment, plasmapheresis and immunoglobuline therapy. Treatment of patients refractory to standard treatment approaches is difficult and results are poor. We describe a 39-year old patient with SLE suffering from grand mal epilepsy due to cerebral vasculopathy with positive lupus anticoagulant, who was refractory to standard treatment modalities. The patient was treated with the anti-CD20 monoclonal antibody rituximab (375 mg/m2 x 4, repeated at weekly intervals). Rituximab applications were delivered in October 2000, March 2001 and October 2001. Since March 2002 she has received maintenance therapy with rituximab 375 mg/m2 every three months. A second female with refractory SLE was treated successfully in April 2002 and receives maintenance therapy every three months. Both patients responded well to rituximab therapy. The first patient showed a major improvement of her clinical condition, and 30 months after the beginning of the rituximab therapy she is free of any symptoms. Inflammation parameters, ANA and lupus anticoagulant declined significantly after the treatment. The clinical condition of the second patient improved dramatically, all inflammation parameters normalized and her circulating immunocomplexes disappeared. In conclusion, rituximab maintenance treatment may be a new effective therapy in SLE.     

Recurrent fractures in an elderly patient with systemic lupus erythematosus

Glucocorticoid‐induced osteoporosis (GIO) is an important problem that remains undertreated, even by rheumatologists. We present a case of an elderly patient with systemic lupus erythematosus diagnosed more than 40 years ago, who suffered from recurrent fractures and attendant complications despite a bone mineral density (BMD) score in the osteopenic range and treatment with bisphosphonates. With improved treatment and outcome of lupus, an increasing number of elderly patients who are susceptible to osteoporotic fractures are expected. This case serves to highlight that rheumatic disease patients on steroids should be screened for GIO, as effective treatment and preventive measures are available. Teriparatide is a promising treatment for patients who have failed bisphosphonate treatment or who are at high risk for fracture. We should also bear in mind that BMD scores alone are not indicative of fracture risk, and other tools such as the WHO‐FRAX (Fracture risk assessment tool), serum vitamin D3 levels and bone turnover markers should be used where appropriate. Other measures including attention to factors that contribute to falls should also be considered, necessitating a multi‐disciplinary approach.     

Recurrent paralytic ileus associated with strongyloidiasis in a patient with systemic lupus erythematosus

We present an interesting case of recurrent paralytic ileus due to strongyloidiasis in a woman who was being treated with corticosteroids and immunosuppressants for systemic lupus erythematosus (SLE). She was also a carrier of human T-cell leukemia virus type I. She had a history of strongyloidiasis 8 years earlier. Recurrent episodes of paralytic ileus due to strongyloidiasis occurred during treatment of her SLE with corticosteroids. Ivermectin was given and improved the symptoms. This case shows that symptomatic strongyloidiasis can be induced in immunocompromised hosts by immunosuppressive therapy. It is important to rule out strongyloidiasis prior to starting immunosuppressive therapy in patients from endemic areas.     

Recurrent autoimmune thrombocytopenia in a patient with systemic lupus erythematosus: treatment with repeated splenectomy!

An unusual case of thrombocytopenia as a manifestation of systemic lupus erythematosus is presented. Management was complicated by regeneration of splenic tissue resulting in recurrent thrombocytopenia and a need for repeated splenectomy.     

Clinical characteristics and outcomes of diffuse alveolar hemorrhage in patients with systemic lupus erythematosus

ObjectiveTo identify the clinical characteristics of diffuse alveolar hemorrhage (DAH) compared to other types of acute diffuse lung infiltration in SLE patients, and the factors associated with mortality in these patients.MethodsWe studied a retrospective cohort including SLE patients with acute diffuse lung infiltration on thoracic CT between January 2004 and August 2014. We divided them into 2 groups, a DAH and a non-DAH group, and compared the clinical characteristics and outcomes in the 2 groups. We also evaluated the risk factors for mortality in SLE patients with diffuse lung infiltration.ResultsOf 47 patients with diffuse lung infiltration, 24 patients (51.1%) satisfied the criteria for DAH and the remaining 23 patients (48.9%) were assigned to the non-DAH group. There were no significant differences between the demographic features of the 2 groups. However, decreased hemoglobin (OR = 3.46; 95% CI: 1.38–8.67; p < 0.01) and C4 (OR = 1.21; 95% CI: 1.03–1.42; p = 0.02) levels, and presence of hypoxia (OR = 23.09; 95% CI: 1.47–365.34; p = 0.03) at the time of diagnosis were associated with SLE-DAH. In addition, severe conditions requiring mechanical ventilation (OR = 64.61; 95% CI: 1.98–2112.02; p = 0.02) were associated with increased mortality, whereas DAH did not increase mortality compared with non-DAH in SLE patients with diffuse lung infiltration.ConclusionsIn SLE patients with acute diffuse lung infiltration, it is important to promptly evaluate the DAH when patients have low levels of hemoglobin or C4, and symptoms of hypoxia. Mortality is associated with severe conditions requiring mechanical ventilation rather than with DAH in patients with diffuse lung infiltration.     

Dyspnea in a patient with systemic lupus erythematosus

A 33-year-old woman with a previous history of systemic lupus erythematosus complained of exerptional dyspnea and pleuritic chest pain accompanied by polyarthritis. Chest-X-rays revealed an elevation of the right hemidiaphragm. We discuss the diagnostic and therapeutic approach.     

Aquired inhibitors of coagulation in a patient with systemic lupus erythematosus and antiphospholipid antibodies: response to rituximab

Hematologic signs of systemic lupus erythematosus are diverse (SLE). Although a delayed coagulation time is anti-phospholipid antibody related, thrombotic events are the usual clinical manifestation. Spontaneous appearance of circulating anticoagulant in the absence of a previous coagulation disorder is secondary to the development of antibodies to factors II, V, VIII, IX, XI, XII,vonWillebrand, and other membrane glucoproteins, all of them uncommon causes (1 case per million persons per year) of life threatening coagulopathies. We report a case of SLE and antiphospholipid antibodies in a woman with a hemorrhagic syndrome, probably caused by multiple antibodies to coagulation factors, unresponsive to steroids and high-dose immunosupressive therapy and a favorable response to rituximab.     

Chronic inflammatory demyelinating polyradiculoneuropathy in a patient with systemic lupus erythematosus and good outcome with rituximab treatment

A patient with chronic inflammatory demyelinating polyradiculoneuropathy and systemic lupus erythematosus arising after rubella vaccination was initially treated with plasmapheresis, corticosteroids and intravenous immunoglobulins, with partial response. After shift to rituximab, most clinical symptoms improved markedly, emphasizing the possible role of this monoclonal antibody in conventional therapy-resistant cases.     

Resistant orbital pseudotumor treated with rituximab in a patient with systemic lupus erythematosus. A case presentation

Ocular manifestations in Systemic Lupus Erythematosus (SLE) are relatively frequent, with a major prevalence of the Keratoconjunctivitis sicca. Nevertheless, the appearance of unilateral exophthalmos secondary to orbital pseudotumor in patients with SLE is extremely rare(1-7), and on occasion it can be refractory to conventional pharmacological treatment (glucocorticoids and immunosuppressants). We present the case of a patient with SLE and orbital pseudotumor refractory to treatment with Cyclophosphamide (CF) and an excellent clinical response, with disappearance of the ophthalmological condition after the beginning of therapy with Rituximab (1g×2), continuing after the infusion of two complete cycles without incidents.     

Primary hyperparathyroidism in a patient with systemic lupus erythematosus

Primary hyperparathyroidism (PHP) is a metabolic illness that results from autonomous secretion of parathyroid hormone and is one of the most common causes of hypercalcemia. We present the case of a 47-year-old female with a previous diagnosis of systemic lupus erythematosus (SLE) in whom clinical (diffuse bone pain, emotional lability, jaw tumor) and laboratory features (calcium= 13.5 mg/dL, phosphate= 1.8 mg/dL, alkaline phosphatase= 3028 U/L, PTH intact= 1472 pg/dL) prompted the diagnosis of PHP secondary to parathyroid adenoma as demonstrated by the anatomopathology. After treatment with calcitonin spray 400 UI per day, IV pamidronate 90 mg/week, and subtotal parathyroidectomy, the patient status improved with normal laboratory tests. This is the second report to describe the coexistence of these two disorders in a single patient. Although the pathophysiology of the association of PHP and SLE is not known, the recognition of this association has a practical implication since the therapeutical strategy is completely different.