Frequency of microangiopathic hemolytic anemia in patients with systemic lupus erythematosus exacerbation: Distinction from thrombotic thrombocytopenic purpura, prognosis, and outcome

Dold, Sylvia; Singh, Ranju; Sarwar, H; Menon, Yamini; Candia, Liliana; Espinoza, Luis R.

doi:10.1002/art.21583

Cited by 27 publications

(12 citation statements)

References 16 publications

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“…Other systemic disorders that may present similarly include such heterogeneous disorders as hemolytic uremic syndrome; malignant hypertension; severe preeclampsia and hemolysis, elevated liver enzymes, and low platelets syndrome; disseminated intravascular coagulation; disseminated malignancy; scleroderma renal crisis; SLE; and APS (4,5). Activation of microvascular endothelial cells is believed to be an important pathogenetic factor in many of these disorders.…”

Section: Differential Diagnosismentioning

confidence: 99%

A 26‐year‐old white man with a systemic lupus erythematosus flare and acute multiorgan ischemia: Vasculitis or thrombosis?

Cherian¹,

Duculan²,

Amigues³

et al. 2011

Arthritis Care & Research

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A 26-year-old white man with a systemic lupus erythematosus (SLE) flare and acute multiorgan ischemia. History of the present illnessThe patient was in his usual state of health until 2 months before admission, when he developed pain and stiffness in his right shoulder, neck, and back after playing softball. His symptoms progressively worsened despite manipulation by a chiropractor, and he developed new pain in his soles. This was diagnosed as plantar fasciitis and he was given a topical steroid injection and a short course of methylprednisolone. This treatment improved his pain, but at its completion, he developed disabling diffuse arthralgias in his shoulders, elbows, knees, and feet, along with fevers, anorexia, and weight loss. Worsening symptoms prompted hospitalization in another institution 3 weeks before admission, where he was febrile up to 103°F and hypertensive (144/100 mm Hg). An extensive evaluation revealed elevated inflammatory markers, including erythrocyte sedimentation rate (ESR; 102 mm/hour), C-reactive protein level (56 mg/liter), positive antinuclear antibodies (ANAs; Ͼ1:640), strongly positive anti-doublestranded DNA (anti-dsDNA) titers, low levels of C4 (9 mg/dl, normal range 16 -38), prolonged prothrombin time (16.2 seconds, normal range 10 -13), prolonged activated partial thromboplastin time (60 seconds, normal range 27-38), and positive lupus anticoagulant (LAC) by dilute Russell's viper venom time. His urine analysis showed proteinuria (0.440 gm protein/gm creatinine), hematuria (11-25 red blood cells [RBCs]/high-power field [hpf]), and pyuria (3-10 white blood cells [WBCs]/hpf). Antibodies to Sm/RNP, Ro/SSA, and La/SSB; anticardiolipin antibodies; antineutrophil cytoplasmic antibodies (ANCAs); and anticyclic citrullinated peptide antibodies were negative, and creatine phosphokinase was normal. An infectious disease evaluation was performed and was negative for all pathogens, including Lyme disease, human immunodeficiency virus, and viral hepatitis. Renal ultrasound and Doppler were performed and were negative for renal pathology and venous thrombosis. He was placed on prednisone 10 mg orally twice daily, losartan 50 mg daily, and aspirin 81 mg daily, and his fevers resolved with some improvement in joint pains. Two weeks before admission, he was evaluated by a rheumatologist who discontinued aspirin in anticipation of a kidney biopsy to rule out lupus nephritis. Laboratory evaluation again revealed persistent proteinuria (2ϩ), hematuria (10 -20 RBCs/hpf), C4 hypocomplementemia (C3 111 mg/dl, C4 9 mg/dl), and strongly positive anti-dsDNA. The patient could not tolerate tapering of prednisone to 15 mg and he stayed at 17.5 mg daily.He presented to our clinic 3 days before admission and his scheduled kidney biopsy. Over the course of the previous week he had developed a few small tender erythematous skin lesions and some skin excoriation on his fingers and palms. He also experienced numbness of his fingertips and bilateral soles for 2 or 3 days. Weight loss of 30 -33 pounds over the cou...

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Section: Differential Diagnosismentioning

confidence: 99%

A 26‐year‐old white man with a systemic lupus erythematosus flare and acute multiorgan ischemia: Vasculitis or thrombosis?

Cherian¹,

Duculan²,

Amigues³

et al. 2011

Arthritis Care & Research

View full text Add to dashboard Cite

show abstract

“…Anaemia of chronic disease is commonly seen in SLE patients, but autoimmune haemolytic anaemia and MAHA are less frequent 6. In view of the presence of haemolytic anaemia in the setting of fragmented red cells in peripheral blood our patient was diagnosed with MAHA.…”

Section: Discussionmentioning

confidence: 91%

“…In view of the presence of haemolytic anaemia in the setting of fragmented red cells in peripheral blood our patient was diagnosed with MAHA. This type of haemolytic process may occur in SLE patients as well in other disorders such as TTP, haemolytic uraemic syndrome, HELLP (haemolysis, elevated liver enzymes, low platelet count) syndrome, disseminated intravascular coagulopathy, vasculitis and antiphospholipid syndrome 6. In general, the prevalence of isolated MAHA in SLE is unknown, but in one study a frequency of 13% was reported 6…”

Section: Discussionmentioning

confidence: 99%

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Nitrofurantoin-induced microangiopathic haemolytic anaemia and thrombocytopaenia in a patient with systemic lupus erythematosus

2012

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Patients with systemic lupus erythematosus (SLE) may develop thrombotic thrombocytopaenic purpura (TTP) or TTP-like illness manifested by microangiopathic haemolytic anaemia (MAHA) and thrombocytopaenia. The distinction between active SLE and TTP is difficult because these entities share similar clinical features. Drug-induced TTP caused by an immune-mediated reaction have been documented for several drugs. Herein, we report a middle-aged Hispanic woman with long-standing SLE, who developed a TTP-like illness characterised by MAHA and thrombocytopaenia after exposure to nitrofurantoin. The patient responded well to plasmapheresis and immunosuppressive therapy and has remained clinically stable after 18 months of follow-up. To our knowledge, this is the first case that reports the association between nitrofurantoin and a TTP-like presentation.

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“…Up to 100 isolated cases of SLE/TMA association were reported in the literature [1,2,[8][9][10][11][12][13][14][15][16][17][18][19][20]. However, an exact frequency cannot be calculated because patients with SLE and HUS/TTP share very similar clinical features [2,21].…”

Section: Discussionmentioning

confidence: 99%

Rapidly progressive lupus nephritis and concomitant thrombotic microangiopathy

et al. 2010

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Although uncommon, thrombotic microangiopathy (TMA) is one of the most serious complications in patients with systemic lupus erythematosus. A 30-year-old black woman admitted to our hospital because of fever, fatigue, 'dark' urine and rapidly progressive renal failure was found to have systemic lupus erythematous and atypical hemolytic uremic syndrome. Kidney biopsy showed WHO class IV lupus nephritis with crescents and TMA. Hemodialysis was initiated for worsening renal failure. The patient was treated with corticosteroids, monthly pulse intravenous Cyclophosphamide, plasmapheresis and Rituximab on a weekly basis for 4 weeks. The patient's blood pressure was aggressively controlled using antihypertensive agents. Despite this extensive therapy, she remained dialysis dependent although hematological parameters returned to normal values.

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Frequency of microangiopathic hemolytic anemia in patients with systemic lupus erythematosus exacerbation: Distinction from thrombotic thrombocytopenic purpura, prognosis, and outcome

Cited by 27 publications

References 16 publications

A 26‐year‐old white man with a systemic lupus erythematosus flare and acute multiorgan ischemia: Vasculitis or thrombosis?

A 26‐year‐old white man with a systemic lupus erythematosus flare and acute multiorgan ischemia: Vasculitis or thrombosis?

Nitrofurantoin-induced microangiopathic haemolytic anaemia and thrombocytopaenia in a patient with systemic lupus erythematosus

Rapidly progressive lupus nephritis and concomitant thrombotic microangiopathy

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