血清阴性多关节炎作为严重的全身性疾病。

Rozin A P, Hasin T, Toledano K, Guralnik L, Balbir-Gurman A

Departments of Rheumatology, Rambam Health Care Campus and Technion, Haifa, Israel.

Neth J Med. 2010 Jun;68(6):236-41.

BACKGROUND

Severe extra-articular disease is associated with high levels of rheumatoid factor (RF ) in patients with seropositive rheumatoid arthritis (RA ) and a poor prognosis. It is said that patients with seronegative rheumatoid arthritis have a more benign course and less destructive disease. We observed several patients with seronegative non-rheumatoid polyarthritis, with aggressive extra-articular systemic disease.

OBJECTIVES

Review of seronegative systemic polyarthritis with clinical presentation of typical cases.

METHODS

Medline search for systemic manifestations of seronegative polyarthritis.

CLINICAL PRESENTATIONS

A 56-year-old woman was admitted to the cardiac intensive care unit with stabbing presternal chest pain aggravated by breathing and progressive dyspnoea, which gradually developed over a period of two weeks with one episode of fever at 38.0 degrees C. She had suffered chronic pain in her buttocks for three years with polyarthralgia and evanescent palmar-plantar rash. Imaging showed bilateral sacroiliitis (HLA B27 negative) and a large pericardial effusion. Extra-articular manifestations of SAPHO syndrome were proposed and she was successfully treated with combined therapy: pulse methylprednisolone, azathioprine, colchicine and prednisone. 2. A 47-year-old woman with psoriatic arthropathy developed high fever with leucocytosis and thrombocytosis and lung infiltrates during exacerbation of her joint disease . She was treated with pulse methylprednisolone followed by corticosteroid tapering, anti-TNF (infliximab) and methotrexate with complete resolution. 3. A 19-year-old man with inflammatory bowel disease developed acute pericarditis with response to 6-mercaptopurine, salazopyrine and prednisone.

RESULTS

We discuss a range of seronegative arthritis diseases with possible systemic manifestations including the main procedures for early diagnosis. Infection, malignancy, hypersensitivity, granulomatous disease and other collagen diseases such as systemic lupus erythematosus should be excluded, but investigations for an underlying disease should not delay early corticosteroid and immunosuppressive therapy.

CONCLUSION

A high level of suspicion of extra-articular disease should always be maintained when treating active seronegative polyarthritis.

背景

在血清阳性类风湿关节炎（RA）患者中，严重关节外疾病与高水平类风湿因子（RF）及不良预后相关。据说血清阴性类风湿关节炎患者病程更良性，疾病破坏性更小。我们观察到数例血清阴性非类风湿性多关节炎患者，伴有侵袭性关节外系统性疾病。

目的

回顾具有典型病例临床表现的血清阴性系统性多关节炎。

方法

通过医学文献数据库检索血清阴性多关节炎的全身表现。

临床表现

一名56岁女性因呼吸时加重的胸骨前刺痛和进行性呼吸困难入住心脏重症监护病房，这些症状在两周内逐渐出现，伴有一次38.0℃发热。她臀部慢性疼痛三年，伴有多关节痛和短暂的掌跖皮疹。影像学显示双侧骶髂关节炎（HLA B27阴性）和大量心包积液。考虑为SAPHO综合征的关节外表现，她接受联合治疗成功：静脉注射甲泼尼龙、硫唑嘌呤、秋水仙碱和泼尼松。2. 一名47岁银屑病关节炎女性在关节疾病加重期间出现高热、白细胞增多、血小板增多和肺部浸润。她接受静脉注射甲泼尼龙治疗，随后逐渐减量使用皮质类固醇，联合抗TNF（英夫利昔单抗）和甲氨蝶呤，症状完全缓解。3. 一名19岁炎症性肠病男性出现急性心包炎，对6-巯基嘌呤、柳氮磺胺吡啶和泼尼松有反应。