Lung findings on thoracic high-resolution computed tomography in patients with ankylosing spondylitis. Correlations with disease duration, clinical findings and pulmonary function testing.

The aim of this study was to identify the spectrum of abnormalities revealed on high-resolution computerized tomography (HRCT) in patients with ankylosing spondylitis (AS), to compare findings with those of plain radiography and pulmonary function testing (PFT), and to look for correlations between lung involvement and AS severity. We prospectively studied 55 consecutive patients with a diagnosis of AS according to the modified New York criteria who attended our department over a period of 2 years. All patients had a detailed rheumatological examination and underwent plain chest radiography, chest HRCT and PFT. HRCT revealed abnormalities in 29 patients (52.7%), whereas plain chest radiography was abnormal in only 2. Abnormalities consisted of interstitial lung disease (ILD) ( n=4), apical fibrosis ( n=5), emphysema ( n=5), bronchiectasis ( n=4), ground glass attenuation ( n=2), and non-specific interstitial abnormalities ( n=26). Only apical fibrosis and bronchiectasis were statistically more frequent with increasing disease duration (significant trend chi(2)test, p=0.0029 and 0.028, respectively). PFT showed a restrictive process in 19 patients (34.5%). No correlation was noted between HRCT and PFT, nor with AS symptomatic and structural severity parameters. However, there was a statistically significant correlation between PFT and AS symptomatic and structural severity parameters. In conclusion,: this study confirms that the chest HRCT of patients with AS showed a great number of abnormalities undetectable by standard X-rays. The high incidence of lung abnormalities emphasizes the importance of excluding such a diagnosis in patients with AS even without respiratory symptoms.

Sausage-like toe and heel pain: value for diagnosing and evaluating the severity of spondyloarthropathies defined by Amor's criteria. A retrospective study in 161 patients.

AIM: To evaluate the frequency of sausage-like toe and talalgia in spondyloarthropathies diagnosed by the 12 items of Amor's criteria; to study the frequency of a diagnosis which would be impossible without these two symptoms; and to study the correlations between Amor's criteria score and the clinical and biological symptoms in the presence of both of these. METHODS: Retrospective study of 161 cases (age, 38.8 +/- 13.1 years; duration, 7.12 +/- 7.3 years; HLA B27, 112). Peripheral involvement is observed in 89 cases (52 cases of pure peripheral form). RESULTS: Eighty cases (group N1) experience talalgia and/or sausage-like toe and 80 do not (group N2). Talalgia concerns 68 patients (42.2%) and sausage-like toe 37 (23.3%); both signs are present in 18. Age, duration, morning stiffness, SR, CRP are similar in both groups. Ankle involvement and oligoarthritis are significantly more frequent in group N1. In 17 cases the presence of talalgia or sausage-like toe are indispensable to the diagnosis (10.6%): 11 undetermined spondyloarthropathies, six psoriatic arthritis. In the N1 group, a positive correlation is present between the score of the 12 items and, respectively, morning stiffness (+ 0.69; P = 0.0001), SR (+ 0.6; P = 0.001 8), CRP (+ 0.59; P = 0.001 8) in patients with sausage-like toe, probably because of the associated oligoarthritis, but neither in patients with talalgia (N1) nor in patients of the group N2. CONCLUSION: Talalgia and sausage-like toe are present respectively in 42.2 and 23.6% of spondyloarthropathies and are indispensable to the diagnosis using Amor's criteria in 10.6% of cases. The sausage-like toe would indicate a more severe spondyloarthopathy because of the synovitis, even though talalgia (pure enthesopathy) does not reflect the inflammatory process.

[Hypokalemic paralysis revealing Sjögren's syndrome associated with auto-immune thyroiditis]. / Quadriparésie hypokaliémique révélant un syndrome de Gougerot-Sjögren associé à une thyroïdite auto-immune.

We report a case of 36-year-old woman, admitted for hypotonic tetraparesis. Laboratory tests revealed severe hypokalaemia, acidosis, hyperchloremia and alkaline urinary pH allowing the diagnosis of distal tubular acidosis. Additional investigations led to the diagnosis of primary Sjögren's syndrome associated with Hashimoto's thyroïditis. The evolution was favorable under potassium citrate alkalinisation, the corticosteroid therapy and hormonal substitution. Based on this observation, the pathogenesis of distal tubular acidosis during auto-immune diseases (Sjögren's syndrome, monoclonal hypergammaglobulinemia, hypothyroidism) was discussed as well as its consequences and management.

Buschke-Ollendorff syndrome.

UNLABELLED: Buschke-Ollendorff syndrome (BOS) is an autosomal dominant disorder characterized by elastin-rich hamartomas and osteopoikilosis. CASE REPORT: In a 21-year-old woman, osteopoikilosis led to the diagnosis of BOS. She had multiple, grouped, buff-colored papules over the thighs and trunk. There was no pain or pruritus associated with the skin lesions. Examination of a biopsy specimen from a papule showed thick uniform collagen fibers and normal numbers of broad interlacing elastic fibers. DISCUSSION: BOS is a rare disease that affects 1/20,000 population. The diagnosis rests on a thorough physical examination and careful examination of radiographs. BOS must be distinguished from other bone abnormalities such as sclerotic bone metastases, particularly when osteopoikilosis is the inaugural manifestation.

Leflunomide-induced toxic epidermal necrolysis in a patient with rheumatoid arthritis.

INTRODUCTION: Leflunomide is an immunomodulating agent with proven efficacy in rheumatoid arthritis. Although its overall safety profile is good, a few cases of toxic epidermal necrolysis have been reported. CASE REPORT: This 36-year-old woman had rheumatoid arthritis that proved refractory to sulfasalazine and methotrexate, which were used successively in combination with symptomatic drugs. Leflunomide was started. A maculopapular rash and a fever developed 2 weeks later. The skin lesions spread rapidly to most of the body, and ulcers of the ocular and oral mucosa appeared. Leflunomide was stopped. Cholestyramine washout and prednisolone (60 mg/day) were given. The skin lesions healed over the next month. Punctate keratitis with keratinization of the cornea led to complete loss of vision. DISCUSSION: The main adverse effects of leflunomide consist of diarrhea, nausea, liver enzyme elevation, hypertension, alopecia, and allergic skin reactions. A few cases of severe skin reactions such as toxic epidermal necrolysis have been reported. They require immediate discontinuation of the drug and a washout procedure to hasten drug elimination from the body. CONCLUSION: Close monitoring for severe skin reactions is in order when using leflunomide.

Sweet's syndrome in Behçet's disease.

UNLABELLED: Few cases of Sweet's syndrome have been reported in patients with Behçet's disease. CASE REPORT: A 47-year-old woman with five year history of oral and genital ulcers that had not been investigated previously was admitted for an eruption of tender, erythematous, vesicle-like papules over the face, neck, palms, and legs. She reported polyarthralgia and weight loss of 6 kg over the previous month. At admission, she had a fever of 38.5 degrees C and conjunctivitis. Joint tenderness to mobilization without evidence of synovitis was noted. Laboratory tests showed inflammation and leukocytosis (12.5x10(9)/L) with 74% neutrophils. The skin biopsy was typical for Sweet's syndrome, and the pathergy test was positive. Glucocorticoid therapy 40 mg/day and colchicine were effective in alleviating the manifestations. DISCUSSION: Overlap exists between the clinical manifestations of Sweet's syndrome and Behçet's disease. Data from the literature suggest that Behçet's disease may be among the conditions that underlie Sweet's syndrome.

[Gastrointestinal bleeding revealing polycythemia. A case report]. / Hémorragie digestive révélatrice d'une polyglobulie. A propos d'une observation.

PURPOSE: Gastrointestinal bleeding, an uncommon complication of polycythemia can be the inaugural sign in exceptional cases. CASE REPORT: A 35-year-old patient was hospitalized for upper gastrointestinal bleeding. Physical examination and laboratory tests led to the diagnosis of polycythemia (Vaquez disease). Gastroscopy showed an hemorrhagic bulber ulcer. Outcome was favorable after chemotherapy and antinuclear treatment. CONCLUSION: Based on this observation and data in the literature we propose that this unusual presentation would suggest a relationship between polycythemia and ulcer disease.

[Sarcoidosis and ankylosing spondylitis. A case report and review of the literature]. / Sarcoïdose et spondylarthrite ankylosante. A propos d'un cas et revue de la littérature.

UNLABELLED: Ankylosing spondylitis is a very uncommon finding in patients with sarcoidosis. Thirteen cases have been reported in the literature. We report a new case. Observation. - A 40-year-old man had inflammatory low back pain since 1983 which formerly responded to nonsteroidal inflammatory drugs (NSAIDs). He developed dyspnea and skin rash in 1993. Physical exam found cervical and lumbar spine stiffness and violaceous and circular lesions on the forehead, nose and right cheek. Laboratory tests showed: ESR at 50 mm, increased serum angiotensin-1-converting enzyme, and negative HLA B27. X-rays showed sacroiliac ankylosis, cervical and lumbar syndesmophytes and cervical facet joint ankylosis. The plain chest x-ray showed an interstitial syndrome. Chest CT scan showed mediastinal adenopathies. Skin biopsy disclosed non-caseating epitheliod and giant-cell granuloma. Outcome was good with steroid therapy but back pain was only improved by NSAIDs. DISCUSSION: - This association raises a diagnostical problem because spine involvement in sarcoidosis can mimic ankylosing spondylitis. It also suggests the hypothesis of a pathophysiological link between the two diseases.