Giant cell arteritis with an erythrocyte sedimentation rate lower than 50.

A high erythrocyte sedimentation rate (ESR) is considered to be a hallmark of giant cell arteritis (GCA) and one of the 1990 American College of Rheumatology classification criteria. We studied 248 patients with GCA to assess the frequency and clinical features of patients with GCA and an ESR <50 mm/h. Ten patients had a low ESR (43.1 +/- 4.9 mm/h) and in the remaining 238, the ESR was > or = 50 mm/h (96.4 +/-23.6). None of the patients with an ESR less than 50 had a completely normal ESR. The spectrum of the disease in both groups was very similar. Both groups required similar corticosteroid therapy and had a similar outcome. The ESR, analysed as a continuous variable, showed a significant positive correlation with other parameters reflecting the acute-phase response such as presence of anaemia, weight loss and fever. We suggest that in patients with a clinical picture compatible with GCA, the use of an ESR > or = 30 mm/h as the main laboratory parameter to consider the possibility of GCA would be enough to arise the suspicion and prevent cases of GCA being missed.

Risk factors and predictive models of giant cell arteritis in polymyalgia rheumatica.

OBJECTIVE: To identify in polymyalgia rheumatica the best set of predictors for a positive temporal artery biopsy and to define predictive models with either a high or low probability of giant cell arteritis (GCA). PATIENTS AND METHODS: Retrospective study of 227 patients, 137 with polymyalgia rheumatica unassociated with arteritis (group A) and 90 with polymyalgia associated with biopsy-proven giant cell arteritis (group B or training set). Data on demographic features, clinical and laboratory abnormalities were collected. Risk factors for arteritis were estimated by nonlinear logistic regressions. Simple predictive models were constructed with those predictors more related to arteritis by multivariable analysis. These models were then tested in group B and in 89 cases of arteritis without polymyalgia rheumatica (group C or test set). RESULTS: The best predictors of arteritis were a new headache odds ratio (OR) 13.6 (95% confidence interval [CI] 4.7 to 39.3); age at onset < 70 years OR 0.11 (CI 0.04 to 0.35); abnormal temporal arteries OR 4.2 (CI 1.3 to 13.7); raised liver enzymes OR 2.9 (CI 1.1 to 7.8), and jaw claudication OR 4.8 (CI 1.0 to 22.7). Amaurosis was only observed in patients with arteritis. Three subsets had a very high risk of arteritis: (1) Patients with recent headache, abnormal arteries, and > or = 70 years at disease onset: sensitivity 44%, positive predictive value (PPV) 93%, likelihood ratio (LR) 20.3; (2) patients with a new headache, jaw claudication, and abnormal arteries: sensitivity 34.4%, PPV 96.9%, LR 47.2; and (3) those, that in addition to the last 3 features, were > or = 70 years of age at disease onset: sensitivity 26.7%, PPV 100%. We could also identify a subset with a very low risk of arteritis constituted by patients < 70 years, without headache, and with clinically normal temporal arteries: sensitivity 1.1%, PPV 1.7%, LR 0.03. In group C or the test set, these four predictive models correctly identified 57.3%, 29.2%, 23.6, and 3.4% of patients, respectively. CONCLUSIONS: In polymyalgia rheumatica it is feasible to identify subsets with a very high likelihood of GCA. Although in some of these subsets the diagnosis of arteritis is almost certain, we suggest that even then it should be confirmed by temporal artery biopsy. By contrast, in those patients with polymyalgia < 70 years and without cranial features of giant cell arteritis, the risk of vasculitis is so low that the biopsy could be initially avoided and the patient treated with low-dose corticosteroids.

Polymyalgia rheumatica without significantly increased erythrocyte sedimentation rate. A more benign syndrome.

BACKGROUND: An erythrocyte sedimentation rate (ESR) of at least 40 mm/h is considered an important requisite for the diagnosis of polymyalgia rheumatica (PMR). However, the relative frequency and clinical features of PMR in patients without a significantly increased ESR are unclear. METHODS: We performed a retrospective study of patients diagnosed as having PMR at the rheumatology divisions of 3 teaching hospitals. The diagnosis of PMR was established, regardless of the ESR, in 201 consecutive patients fulfilling the following criteria: (1) age 50 years or older, (2) severe proximal pain for more than 1 month in at least 2 of 3 areas: neck, shoulder, and/or pelvic girdles, and (3) rapid resolution of the syndrome while taking low-dose prednisone. Patients with giant cell arteritis were previously excluded from the study. The frequency and clinical features of patients with PMR and an ESR lower than 40 mm/h were analyzed. A comparative study between these patients and those with high ESRs was performed. RESULTS: An ESR lower than 40 mm/h was found in 41 patients (20.4%). These patients were younger (P = .02), were more frequently men (P = .006), and experienced a lower frequency of fever (P = .003) and weight loss (P = .07). Furthermore, these patients were characterized by an absence of anemia (P = .002) and a lower frequency of abnormal protein electrophoresis results (P < .001). Otherwise, their clinical syndrome, response to therapy, and frequency of relapses were similar to those of patients with classic PMR. In the entire population of 201 patients, the ESR was related to the length of treatment, number of areas involved, presence of fever, weight loss, and laboratory test result abnormalities, but it was unrelated to the duration of the illness prior to diagnosis. CONCLUSIONS: It is not uncommon to find a patient with PMR with an ESR lower than 40 mm/h. This syndrome is more frequent in men and it is clinically less severe than the classic form of PMR. Its recognition will allow these patients to benefit from an effective treatment with low-dose corticosteroids.

Acute febrile toxic reaction in patients with refractory rheumatoid arthritis who are receiving combined therapy with methotrexate and azathioprine.

OBJECTIVE: To assess the frequency and clinical features of an acute febrile toxic reaction (AFTR) in patients with refractory rheumatoid arthritis (RA) receiving combined therapy with methotrexate (MTX) and azathioprine (AZA). METHODS: A cohort of 43 RA patients being treated with MTX/AZA combination therapy were studied. In all of them, RA had been refractory to single-therapy disease-modifying antirheumatic drugs. We analyzed the frequency and clinical features of AFTR, which consisted mainly of the development of fever, leukocytosis, and cutaneous leukocytoclastic vasculitis when AZA was added to the MTX regimen. RESULTS: Four of the 43 patients (9.3%) who had been receiving long-term, well-tolerated treatment with MTX (mean +/- SD 375.5 +/- 159.5 days, range 227-561 days) developed AFTR shortly (mean +/- SD 25.7 +/- 13.6 days, range 17-46 days) after the addition of AZA to the regimen. The AFTR resolved rapidly (3 +/- 1.4 days) after discontinuation of AZA and MTX. In 2 cases, rechallenge with AZA and MTX was linked to a new flare of AFTR. CONCLUSION: The knowledge of this side effect is particularly important because it mimics a severe infectious complication related to immunosuppressive therapy, and because rechallenge can produce severe toxicity. Most of the new combined therapies for RA do not seem to be more toxic than single-drug treatment. Nevertheless, clinicians should be aware of a possible increase in side effects due to drug interactions or some other unidentified mechanism.

Mononeuritis multiplex in systemic lupus erythematosus: response to pulse intravenous cyclophosphamide.

Severe mononeuritis multiplex in systemic lupus erythematosus (SLE) is quite rare and almost always accompanied by evidence of active disease in other organs, although occasionally it may be the presenting feature of the disease. We describe here two patients with SLE who presented a severe mononeuritis multiplex secondary to a necrotizing vasculitis which respond successfully to therapy with intravenous cyclophosphamide, and review the literature of similar cases.

[Giant cell arteritis. A study of 191 patients]. / Arteritis de células gigantes. Estudio de 191 pacientes.

BACKGROUND: The aim of the present was to study the clinical features of a wide series of patients with giant cell arteritis (GCA) diagnosed with accurate criteria and to evaluate the sensitivity of the criteria proposed by the ACR for classification of GCA. METHODS: A retrospective analysis of 191 patients with GCA, 184 of whom were diagnosed by biopsy and 7 due to their clinical manifestations was carried out. RESULTS: The age was 73 +/- 7 years with the most frequent symptoms being headache (87%), abnormalities in the temporal arteries (75%), general malaise (60%), rheumatic polymyalgia (49%) and mandibular claudication (40%). The frequency of GCA was equal in both genders although the most complex syndrome was observed in women with a greater frequency of polymyalgia (p < 0.005), jaw claudication (p < 0.01) and anemia (p < 0.01). The patients with polymyalgia were characterized by a predominance of the polymyalgic syndrome in the initial phases and a higher frequency of amaurosis. Out of 47 patients with amaurosis, 23 remained with permanent unit or bilateral blindness. Unilateral biopsy of the temporal artery was diagnosed in 91% of the cases (CI 95%; 86 to 95%) increasing to 96.3% (CI 95%; 92 to 98%) on biopsy of both arteries. Ninety-eight percent of the patients (CI 95%; 95 to 99%) had 3 or more GCA criteria for classification as GCA. CONCLUSIONS: The clinical manifestations of giant cell arteritis in Spain, with the exception of an equal frequency in both sexes, are similar to that described in other series of patients selected with strict criteria. The present data confirm the sensitivity of the criteria proposed by the ACR for the classification of giant cell arteritis although its application does not avoid the need for temporal artery biopsy for diagnosis. Unilateral biopsy is usually suffice in most of the cases.

Acute reversible hypoxemia in systemic lupus erythematosus: a new syndrome or an index of disease activity?

In 1991, Abramson et al reported a new syndrome of acute reversible hypoxemia (ARH) in patients with severe SLE (systemic lupus erythematosus). This syndrome was characterized by an unexplained abnormal value of arterial blood gases (ABG) without obvious parenchymal lung disease, and a good response to high-dose corticosteroid therapy. After we became aware of this entity, four of 16 patients admitted to our unit because of a SLE flare presented respiratory symptoms and abnormal ABG consistent with ARH. In none of our patients were the pulmonary manifestations a prominent clinical feature of the disease. Furthermore, in two of them, treatment with high-dose aspirin and moderate to low doses of corticosteroids was sufficient to improve the pulmonary manifestations, but not to control the systemic activity of the disease. Therefore, we believe that this new pulmonary finding more than a clinically independent syndrome represents an index of disease activity in patients with SLE.

Malignant ventricular arrhythmia in systemic sclerosis controlled with an implantable cardioverter defibrillator.

The prognosis of systemic sclerosis (SSc) is poor, and a significant number of patients suffer sudden death, probably related to malignant ventricular arrhythmias for which there is no reliable drug treatment. We describe a patient with SSc who had 2 documented episodes of ventricular fibrillation, that reverted after electrical defibrillation. An electrophysiological study performed after 3 weeks of oral loading with amiodarone 1200 mg/day, demonstrated the induction of unstable sustained syncopal ventricular tachycardia. She has been successfully controlled with the implant of a 3rd generation implantable cardioverter defibrillator. We suggest that this treatment should be seriously considered in those patients with SSc and malignant ventricular arrhythmias unresponsive to drug therapy.