Combined ANCA-associated vasculitis and lupus syndrome following prolonged use of hydralazine: a timely reminder of an old foe

Sangala, Nicholas; Lee, Richard W.; Horsfield, Catherine; Goldsmith, David

doi:10.1007/s11255-009-9627-9

Cited by 10 publications

(7 citation statements)

References 12 publications

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“…Cases in which splenomegaly was found suggest the potential for neutrophil sequestration (9, 20–22, 27, 36). Evidence for peripheral destruction of neutrophils was supported by the presence of antibodies to neutrophil antigens and by the findings of hypercellularity on bone marrow biopsy (7, 13, 37). …”

Section: Discussionmentioning

confidence: 98%

“…Ahmed et al report an 18 year old patient who developed moderate neutropenia, c-ANCA with specificity to PR3, high-titer ANA, and constitutional symptoms while taking minocycline for acne (12). Sangala et al described a patient with SLE taking hydralazine who developed biopsy-proven, pauci-immune glomerulonephritis and pancytopenia (13). The neutropenia was attributed to a lupus-like syndrome and resolved with cessation of hydralazine.…”

Section: Literature Reviewmentioning

confidence: 99%

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Antineutrophil Cytoplasmic Antibodies, Autoimmune Neutropenia, and Vasculitis

Grayson

Sloan

Niles

et al. 2011

Seminars in Arthritis and Rheumatism

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Objectives Reports of an association between antineutrophil cytoplasmic antibodies (ANCA) and autoimmune neutropenia have rarely included cases of proven vasculitis. A case of ANCA-associated vasculitis (AAV) with recurrent neutropenia is described and relevant literature on the association between ANCA, neutropenia, and vasculitis is reviewed. Methods Longitudinal clinical assessments and laboratory findings are described in a patient with AAV and recurrent episodes of profound neutropenia from December 2008 – October 2010. A PubMed database search of the medical literature was performed for papers published from 1960 through October 2010 to identify all reported cases of ANCA and neutropenia. Results A 49 year-old man developed recurrent neutropenia, periodic fevers, arthritis, biopsy-proven cutaneous vasculitis, sensorineural hearing loss, epididymitis, and positive tests for ANCA with specificity for antibodies to both proteinase 3 and myeloperoxidase. Antineutrophil membrane antibodies were detected during an acute neutropenic phase and were not detectable in a post-recovery sample, whereas ANCA titers did not seem to correlate with neutropenia. An association between ANCA and neutropenia has been reported in 74 cases from 24 studies in the context of drug/toxin exposure, underlying autoimmune disease, or chronic neutropenia without underlying autoimmune disease. In these cases, the presence of atypical ANCA patterns and other antibodies were common; however, vasculitis was uncommon and when it occurred was usually limited to the skin and in cases of underlying toxin exposure. Conclusions ANCA is associated with autoimmune neutropenia, but systemic vasculitis rarely occurs in association with ANCA and neutropenia. The interaction between neutrophils and ANCA may provide insight into understanding both autoimmune neutropenia and AAV.

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Section: Discussionmentioning

confidence: 98%

Section: Literature Reviewmentioning

confidence: 99%

Antineutrophil Cytoplasmic Antibodies, Autoimmune Neutropenia, and Vasculitis

Grayson

Sloan

Niles

et al. 2011

Seminars in Arthritis and Rheumatism

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“…4 A severe case of hydralazine-induced lupus and antineutrophil cytoplasmic antibody vasculitis responded to pulsed methylprednisolone, plasma exchange, and hemodialysis. 12 Three of 21 patients in Speirs' hydralazine-induced lupus report experienced central nervous system signs and symptoms, 6 but this is an unusual observation. Severe organ involvement is exceedingly rare, but pericardial tamponade 1,13 and fatality with pneumonitis has been reported.…”

Section: Uncommon Clinical Featuresmentioning

confidence: 99%

Hydralazine‐Induced Lupus Erythematosis

Handler

2011

J of Clinical Hypertension

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A 65-year-old woman was seen in our hypertension clinic following recovery from hydralazine-induced lupus erythematosis. She had been taking antihypertensive medication for 15 years and was evaluated by the rheumatology department a year prior to her hypertension consultation for joint pain and swelling associated with positive serologies.A month prior to her rheumatology appointment she developed acute pain and swelling in the left fifth proximal interphalangeal joint (PIP), followed by painful swelling of the fourth and fifth PIP joints allowing minimal flexion. Subsequent joint involvement progressed to the right fifth PIP joint, bilateral wrists, right shoulder, bilateral knees, bilateral ankles, and both forefeet. Laboratory tests were significant for a diffuse pattern antinuclear antibody 1:2560 and a low positive rheumatoid factor of 17 IU ⁄ mL (normal <11 IU ⁄ mL). A complete blood cell count and renal function were normal. She denied fever, rash, pleuritic chest pain, or shortness of breath.Rheumatology examination noted swelling and tenderness of the left third, fourth, and fifth PIP joints with dactylitis, swelling and tenderness of the right fifth PIP, and bilateral tender wrists. Additional serologies revealed antidouble stranded deoxyribonucleic acid antibody (anti-ds DNA ab) 15 IU ⁄ mL (normal <25.0 IU ⁄ mL), antihistone antibody 1.8 UOM ⁄ U (normal <1.0 UOM ⁄ U), C3 complement 153 mg ⁄ dL (normal 90-180 mg ⁄ dL), C4 complement 21 mg ⁄ dL (normal 10-40 mg ⁄ dL), and Westergren erythrocyte sedimentation rate 43 mm ⁄ h (normal 0-33 mm ⁄ h). Her 4-drug antihypertensive drug regimen included hydralazine 50 mg twice a day for 5 years.Hydralazine was discontinued and azulfidine was initiated and advanced to 1000 mg twice a day with meloxicam 15 mg daily. Painful joints rapidly abated and 6 months later azulfidine was discontinued without a recurrence of her inflammatory arthritis. Losartan, amlodipine, and atenolol were continued. When chlorthalidone was substituted for furosemide, elevated blood pressures off of hydralazine became controlled.

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“…Hydralazine can rarely induce an antineutrophil cytoplasmic antibody (ANCA)-positive vasculitis and less than 80 such cases have been reported hitherto. 1,2 Here, we report the case of an elderly patient who was diagnosed with hydralazine-induced ANCA vasculitis after taking hydralazine for 5 years.…”

Section: Sirmentioning

confidence: 99%