Journal Information
Vol. 52. Issue 3.
Pages 172-173 (March 2016)
Vol. 52. Issue 3.
Pages 172-173 (March 2016)
Letter to the Editor
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Antineutrophil Cytoplasmic Antibodies (ANCA)-Negative Vasculitis in a Patient With Alpha-1-Antitrypsin Deficiency
Vasculitis con Anticuerpos anticitoplasma de neutrófilos (ANCA) negativos en paciente con déficit de alfa-1 antitripsina
Juan Marco Figueira Gonçalves
Corresponding author

Corresponding author.
, Rosella D’amato
Servicio de Neumología, Hospital Universitario Nuestra Señora de la Candelaria, Santa Cruz de Tenerife, Tenerife, Spain
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To the Editor

We report the case of a 62-year-old man, former smoker of 20 pack-years, with a history of arterial hypertension, diabetes mellitus and previous ictus with no neurological sequelae. He was referred from the respiratory medicine clinic with dyspnea on moderate effort (mMRC 2). Lung function tests showed FEV1/FVC 47%; FEV1 1.4 l (44%); FVC 3.3 l (82%); VR 4.4 l (182%); TLC 8.0 l (119%); DLCO 49% and KCO 59%. Chest computed tomography showed severe panacinar emphysema, primarily in the lower lobes. Severe alpha-1-antitrypsin (AAT) deficiency (28.4mg/dl) associated with ZZ phenotype was observed. A diagnosis of COPD with severe airflow obstruction and type ZZ alpha-1-antitrypsin deficiency (AATD) was made, and after abdominal ultrasound confirmed chronic liver disease, the decision was made to administer replacement AAT. Before initiation of treatment, the patient had an episode of epistaxis associated with purplish lesions in the lower limbs, tending to converge, with no blanchability on diascopy. Pathology study results showed leukocytoclastic vasculitis in small and medium caliber vessels, associated with elevated IgA (754mg/dl), microhematuria and proteinuria suggestive of nephritis. Together, these signs were consistent with a diagnosis of adult Henoch–Schönlein purpura (HSP). ANCA antibodies (MPO<0.8IU/ml and anti-PR3<0.4IU/ml) and ENA were negative; ANA were positive with a titer of 1/160 in a fine speckled pattern. On the basis of this diagnosis, treatment began with oral corticosteroids (0.5mg/kg/day) in a tapering regimen.

One month after beginning this treatment, the patient suffered a fall at home and injured his left arm, with subsequent development of diffuse arthralgia and asthenia. Magnetic resonance imaging revealed cellulitis, arthritis and synovitis in the distal radioulnar and carpometacarpal joints of the upper left limb. Synovial biopsy and fluid culture were performed, confirming synovitis with positive multi-resistant Pseudomonas aeruginosa culture. Despite the administration of wide-spectrum antibiotics and systemic corticosteroids, the patient developed multiorgan failure and died in the intensive care unit.

A review of AATD and concomitant necrotizing vasculitis shows that microscopic polyangiitis and Wegener's granulomatosis are the most common forms, while HSP is an unusual manifestation. The mean age for presentation is generally around 48 years, and there is no prevalence with regard to sex. All cases present skin involvement, along with kidney or joint disease. In cases of HSP, hepatic cirrhosis is usually observed.1,2

Our patient presented the foregoing conditions, further complicated by severe sepsis due to cellulitis and arthritis of the wrist. Evidence shows that AATD patients can occasionally develop necrotizing panniculitis induced by injury.3 In our case, the histological diagnosis could not be confirmed, since a synovial biopsy, rather than a skin biopsy, was obtained, but the clinical signs and symptoms and progress were consistent with this diagnosis.

A. Mohammad, M. Segelmark.
Primary systemic vasculitis with severe α1-antitrypsin deficiency revisited.
Scand J Rheumatol, 43 (2014), pp. 242-245
P. Mazodier, A.N.Y. Elzouki, M. Segelmark, S. Eriksson.
Systemic necrotizing vasculitides in severe alpha1-antitrypsin deficiency.
QJM, 89 (1996), pp. 599-611
R. Valverde, B. Rosales, F.J. Ortiz-de Frutos, J.L. Rodríguez-Peralto, P.L. Ortiz-Romero.
Alpha-1-antitrypsin deficiency panniculitis.
Dermatol Clin, 26 (2008), pp. 447-451

Please cite this article as: Gonçalves JMF, D’amato R. Vasculitis con Anticuerpos anticitoplasma de neutrófilos (ANCA) negativos en paciente con déficit de alfa-1 antitripsina. Arch Bronconeumol. 2016;52:172–173.

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