Cutaneous vasculitis associated with severe bacterial infections. A study of 27 patients from a series of 766 cutaneous vasculitis.

OBJECTIVES: To assess the clinical spectrum of severe bacterial infections presenting as cutaneous vasculitis (CV) in a defined population. METHODS: Unselected series of 766 patients with CV diagnosed at a single university referral center. RESULTS: An underlying severe bacterial infection was diagnosed in 27 (22 men/5 women; mean age ± standard deviation [SD]: 53 ± 18 years) of 766 cases presenting with CV (3.5%). These infections were: pneumonia (n=8), endocarditis (n=6), meningitis (n=4), intra-abdominal infections (n=3), septic arthritis (n=2), septicaemia (n=2), septic bursitis (n=1), and urinary tract infection (n=1). All the patients were admitted for suspected CV. The median delay from admission to the diagnosis of infection was 4 days. A typical palpable purpura without relevant visceral vasculitic involvement was the main clinical manifestation. Patients with severe bacterial infections were older, with male predominance, had more frequently fever, constitutional symptoms, focal infectious features, and leukocytosis with left shift and anaemia than the remaining patients with CV. Although antibiotics were prescribed in all the patients, seven also required the use of low-dose corticosteroids to achieve complete resolution of the cutaneous lesions. Most patients experienced full recovery but two of them underwent prosthetic cardiac valve replacement, and another two died due to infection-related complications. CONCLUSIONS: CV may be the presenting manifestation of a severe underlying bacterial infection. Physicians should keep in mind this fact to make an early diagnosis of infection and, consequently, prevent life-threatening complications.

Applicability of the 2006 European League Against Rheumatism (EULAR) criteria for the classification of Henoch-Schönlein purpura. An analysis based on 766 patients with cutaneous vasculitis.

OBJECTIVES: In 2006 the European League Against Rheumatism (EULAR) proposed new classification criteria for Henoch-Schönlein purpura (HSP). We aimed to establish the applicability of these criteria in patients with primary cutaneous vasculitis (CV). We also compared these criteria with previously established classification criteria for HSP. METHODS: A series of 766 (346 women/420 men; mean age 34 years) consecutive unselected patients with CV was assessed. One hundred and twenty-four of them with secondary CV or with CV associated with other well defined entities were excluded from the analysis. The 2006 EULAR criteria for HSP were tested in the remaining 642 patients with primary CV. Two sets of criteria for HSP were used for comparisons: a) the 1990 American College of Rheumatology (ACR-1990), and b) the ACR modified criteria proposed by Michel et al. in 1992 (Michel-1992). RESULTS: 451 (70.2%) of 642 patients were classified as having HSP according to the EULAR-2006 criteria, 405 (63.1%) using the ACR-1990 criteria, and 392 (61.1%) by the Michel-1992 criteria. However, only 336 patients (52.3%) met at the same time the EULAR-2006 and the ACR-1990 criteria, and only 229 patients (35.7%) fulfilled both the EULAR-2006 and Michel-1992 criteria. It is noteworthy that only 276 (43%) patients met the three set of criteria. Children fulfilled all the sets of criteria more commonly than adults (215 [66.6%] of 323 vs. 61 [19%] of 319, respectively; p<0.0001). CONCLUSIONS: According to our results, the EULAR-2006 criteria show low concordance with previous sets of classification criteria used for HSP.

Non-infectious aortitis: a report of 32 cases from a single tertiary centre in a 4-year period and literature review.

OBJECTIVES: Non-infectious aortitis often presents with non-specific symptoms leading to inappropriate diagnostic delay. We intend to describe the clinical spectrum and outcome of patients with aortitis diagnosed at a single centre. METHODS: We reviewed the clinical charts of patients diagnosed with non-infectious aortitis between January 2010 and December 2013 at the Rheumatology Division from a 1.000-bed tertiary teaching hospital from Northern Spain. The diagnosis of aortitis was usually based on FDG-PET-CT scan, and also occasionally on CT or MRI angiography or helical CT-scan. RESULTS: During the period of assessment 32 patients (22 women and 10 men; mean age 68 years [range, 45-87]) were diagnosed with aortitis. The median interval from the onset of symptoms to the diagnosis was 21 months. FDG-PET CT scan was the most common tool used for the diagnosis of aortitis. The underlying conditions were the following: giant cell arteritis (n=13 cases); isolated polymyalgia rheumatica (PMR) (n=11); Sjögren's syndrome (n=2), Takayasu arteritis (n= 1); sarcoidosis (n=1), ulcerative colitis (n=1), psoriatic arthritis (n=1), and large-vessel vasculitis that also involved the aorta (n=2). The most common clinical manifestations at diagnosis were: PMR features, often with atypical clinical presentation (n=23 patients, 72%); diffuse lower limb pain (n=16 patients, 50%); constitutional symptoms (n=12 patients, 37%), inflammatory low back pain (n=9 patients, 28%) and fever (n=7 patients, 22%). Acute phase reactants were increased in most cases (median erythrocyte sedimentation rate 46 mm/1st hour, and a median serum C-reactive protein 1.5 mg/dL). CONCLUSIONS: Aortitis is not an uncommon condition. The diagnosis is often delayed. Atypical PMR features, unexplained low back or limb pain, constitutional symptoms along with increased acute phase reactants should be considered 'red flags' to suspect the presence of aortitis.

Henoch-Schönlein purpura nephritis and IgA nephropathy: a comparative clinical study.

OBJECTIVES: Henoch-Schönlein purpura nephritis (HSPN) and IgA nephropathy (IgAN) are related syndromes. In the present study we aimed to compare the clinical characteristics and outcome of a large and unselected series of patients diagnosed as having HSPN and IgAN. METHODS: Comparative study of a wide and unselected population of HSPN (142 patient) and IgAN (61 patients) from a teaching hospital of Northern Spain. RESULTS: All of the following comparisons were expressed between HSPN vs. IgAN, respectively. HSPN patients were younger (30.6±26.4 vs. 37.1±16.5 years, p<0.001). Precipitating events, usually an upper respiratory tract infection and/or drug intake, were more frequently observed in HSPN (38% vs. 23%, p=0.03). Extra-renal manifestations were also more common in HSPN than in IgAN; skin lesions (100% vs. 1.8%; p<0.001), gastrointestinal (62% vs. 7.4%; p<0.001), and joint involvement (61.3% vs. 3.6%; p<0.001). However, nephritis was less severe in HSPN, renal insufficiency (25% in HSPN vs. 63.4% in IgAN; p<0.001), nephrotic syndrome (12.5%, vs. 43.7%; p<0.001), and nephritic syndrome (6.8% vs. 10.7%; NS). Leukocytosis was more frequent in HSPN (22.5% vs. 8.2%; p=0.015) and anaemia in IgAN (12.7% in HSPN vs. 36% in IgAN, p<0.001). The frequency of corticosteroid (79.6% vs. 69%; NS) and cytotoxic drug (19% vs. 16.5%, NS) use was similar. The frequency of relapses was similar (38.6% in HSPN vs. 36.3% in IgAN). After a median follow-up of 120.8 (IQR; 110-132) months in HSPN and 138.6 (IQR; 117-156) in IgAN, requirement for dialysis (2.9% vs. 43.5%; p<0.001), renal transplant (0% vs. 36%, p<0.001) and residual chronic renal insufficiency (4.9% vs. 63.8%; p<0.001) was more frequently observed in patients with in IgAN. CONCLUSIONS: HSPN and IgAN represent different syndromes. IgAN has more severe renal involvement while HSPN is associated with more extra-renal manifestations.