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病例漫谈100:成功治疗原发性肾上腺和脊柱结核病例的三年随访 [复制链接]

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Tuberculosis (TB)remains a major public health problem, ranking as the second leading cause ofdeath from an infectious disease worldwide, after the human immunodeficiencyvirus (HIV). World Health Organization (WHO) reported more than 2 billionpeople, equal to one-third of the world’s population, are infected with TBbacilli and 1.3 billion people die from TB. In 2011 alone, WHO reported morethan 9 million new cases of TB and 1.4 million TB deaths worldwide.Extrapulmonary tuberculosis (EPTB) is increasing and accounts for one in fiveof the new diagnosed TB patients. In many part of the world, TB remains one ofthe leading causes of adrenal insufficiency and it is vital to identify theinfectious cause for Addison disease to prevent patient from developinglife-threatening adrenal crisis. Adrenal TB is characterized by a peculiarclinical and radiological pattern, and its diagnosis is difficult to obtaineven after biopsy. Approximately 50% of skeletal TB involves the spine, mostcommonly affecting the lower thoracic and upper lumbar levels. We here report asuccessfully treated unusual case of primary combined adrenal and spinal TBcomplicated with chronic adrenal insufficiency, with a combination of anti-TBdrugs and steroid therapy. We also present a three years follow up for ourpatient showing significant changes in the adrenal glands radiologicalfindings.

结核病(TB)一直是一个重大的公共卫生问题,在全球传染性疾病中致死率位居第二,仅次于人类免疫缺陷病毒(HIV)。世界卫生组织(WHO)报告称已有20多亿人受结核杆菌感染,相当于世界总人口的三分之一,13亿人因结核病死亡。  WHO报道称,仅2011年,全球就有超过900万的新增结核病人,同时有140万人死于结核病。肺外结核(EPTB)病患者仍在持续增加,占新增结核病患者的五分之一。在世界许多地区,结核病仍是导致肾上腺功能不全的主要原因之一。明确艾迪生病的传染原因从而控制患者病情,不至其发展到危及生命的肾上腺疾病是非常重要的。肾上腺结核具有独特的临床和影像学特征,即使是活检也很难做出诊断。50%的骨骼结核病涉及脊柱,通常多发于下胸和上腰椎段。本文报道了一例患原发性肾上腺和脊柱结核,并发慢性肾上腺功能不全的患者,接受抗结核药物和类固醇治疗成功的罕见病例。我们对患者进行了3年随访,肾上腺的影像学检查结果呈现出明显变化。


Patient and observation

A 36 year old malefrom Asian origin presented on 13/10/2009 with hyperpigmentation and darkeningof the skin that had been developed for previous 5 months accompanied byfatigue for a period of more than a year with lumbar back pain. During hisphysical examination, he had a core body temperature of 36°C , a pulse 84 beatsper minute, a respiratory rate 20 breaths per minute and a blood pressure of100/60 mmHg. He had mild pallor, but no jaundice or cyanosis. Skin showedgeneralized hyperpigmentation with prominent gingival and buccal mucosadarkening. There was no significant peripheral lymphadenopathy. Thyroid glandwas not enlarged. His abdomen was soft and flat with no organomegaly. Chest andcardiac examinations revealed no abnormalities. Laboratory findings included ared blood cell count of 4.51 × 10 12 /L; hemoglobin 11.1 g/dl; whiteblood cell count 16.31 × 10 9 /L; (neutrophils 61.6%; lymphocytes13.1%; monocytes, 7.0%; eosinophils, 0.1%; basophils, 0.7%); platelet count 308× 10 9 /L; high erythrocyte sedimentation rate (42 mm/h), highC-reactive protein (14.88mg/L), low-serum sodium (129 mmol/L), high-serumpotassium (5.4 mmol/L), low serum chloride (92 mmol/L), blood glucose level(118 mg/dl), normal kidney function, normal liver function, low-serum cortisol8 am (2.9 µg/dL), 4 pm (2.6 µg/dL), TB IgM antibody (-ve), TB IgG antibody(+ve) and positive purified protein derivative test (PPD). A Chest x-rayrevealed no abnormalities. A Thoracic lumbar plain film revealed narrowing ofthe L1- L2 space with irregularity and osteolysis of the endplates suggestingthe possibility of lumbar spine TB (Figure 1-A). An Abdominal CT imagingrevealed bilateral adrenal mass like enlargement with irregular enhancement andlesion like changes without calcification (Figure 1-b-d). Based on the clinicalpresentation, laboratory findings of positive IgG anti-TB antibodies, positivePPD test, High ESR, low serum cortisol, low sodium level and high potassiumlevel, beside the radiological findings of spinal and adrenal involvement, thediagnosis of combined primary adrenal and spinal TB with adrenal insufficiencywas highly suggested.

患者和观察

一例亚洲男性患者,36岁,于20091013日首次就诊,5个月前即出现色素沉着和皮肤黑变,且有长达1年多的腰背部疼痛。体格检查发现患者体温36°C,脉搏84/分,呼吸频率20/分,血压100 / 60 mmHg,有轻度苍白或紫绀,无黄疸,皮肤色素沉着过度,牙龈突出,颊粘膜呈黑色,未发现外周淋巴结和甲状腺明显肿大。患者腹部平软,无脏器肿大,胸部和心脏检查正常。化验结果包括红细胞计数4.51 × 10 12 /L、血红蛋白11.1 g/L、白细胞计数16.31 × 10 9 /L;(中性粒细胞61.6%;淋巴细胞13.1%;单核细胞7.0%、嗜酸性粒细胞0.1%、嗜碱性粒细胞0.7%);血小板计数308 × 10 9/L;红细胞沉降率高(42mm/h)、高敏C反应蛋白(14.88mgL),血钠较低(129 mmol/L)、血清钾浓度较高(5.4mmol/L),血氯较低(92 mmol/L),血糖水平(118mg/dL),肾功能正常,肝功能正常,血清皮质醇低(早上8点为2.9µg/dL,下午4点为2.6µg/dL),结核抗体IgM-ve)、结核抗体IgG+ ve)和纯化蛋白衍生物(PPD)试验呈阳性。胸部X线片正常。胸腰椎平片显示L1- L2之间呈现不规则缩小,终板呈现骨质溶解,这些都暗示了患者可能患有腰椎结核(图 1-A)。腹部CT显示双侧肾上腺肿块同样呈现无规则肿大,同时发生无钙化病变(图 1-B)。根据患者的临床表现、化验检查中IgG抗结核抗体呈阳性、PPD试验呈阳性,高血沉,低血清皮质醇、低钠高钾,以及脊髓和肾上腺相关的影像学表现,可以诊断患者很可能患有原发性肾上腺和脊髓结核,并发肾上腺功能不全。


Based on thisdiagnosis, the patient started anti-TB drugs in the form of (isoniazid,rifampicin, ethambutol, pyrazinamide) in combination with vitamin B6 tabletsand steroid therapy in the form of prednisolone (7.5 mg/day) in two divideddose of 5 mg in the morning 8 am and 2.5 mg afternoon at 4 pm. He was calledfor clinical follow up every 6 months and all the necessary investigations wereperformed each time.

基于以上诊断,患者同时服用了抗结核药物(异烟肼、利福平、乙胺丁醇、吡嗪酰胺),维生素B6片和类固醇类药物(泼尼松龙(7.5mg/天):早上85mg,下午42.5mg)进行治疗。患者每6个月接受一次临床随访,并进行了所有必需的检查。


In the recent followup of our patient in 2012, he was without clinical symptoms and all thelaboratory investigations were nearly normalized. The anti TB drugs werestopped but steroid therapy in the form of prednisolone is continued with samedose as earlier. The thoracic lumbar plain film taken later in 2012, that wehave presented in the article demonstrated nearly the same finding as shown inthe earlier film taken during the initial presentation (Figure 1-e). A CT scanof his adrenal glands documented a relevant reduction of the bilateral adrenalmasses and the adrenal lesions were significantly reduced than the earlier CTfilm along with diminished degree of enhancement (Figure 1-f-h), however it isnot conclusive about the restoration of its normal adrenal function.

2012年,最近一次的随访中,患者已无临床症状,所有化验结果趋于正常。患者已停用抗结核药物,但仍服用类固醇类药物泼尼松龙,剂量保持不变。 2012年,患者拍摄了胸腰椎平片,文中展示了此次检测结果,并证实与早期拍摄的胸腰椎平片结果具有一致性(图 1-e)。与早期检查结果相比,此次CT扫描结果发现患者双侧肾上腺肿块和肾上腺病变明显减少,病变增强程度也随之减弱(图1-f-h),然而这并不能证明肾上腺功能已恢复正常。

Figure1: Pre-treatment (a) and post treatment (e) lateral lumbar plain film showingfusion and irregular osteolytic destruction of the L1, L2 vertebral bodies (arrow).Pre-treatment (b, c, d) and Post-treatment (f, g, h) CT- abdomen of differentphase showing bilateral enlargement of adrenal gland (upper arrow) withirregular enhancement and lesion like changes and decrease in adrenal glandsize (lower arrow) and significant reduction in adrenal lesions respectively
1:治疗前(a)和治疗后(e)腰椎侧位平片显示L1L2椎体出现融合及不规则的溶骨性损伤(箭头所示)。治疗前(b,c, d)腹部不同部位CT图像显示双侧肾上腺扩大(上箭头),且呈现不规则的增强趋势;治疗后(f,g, h)腹部不同部位CT图像显示肾上腺大小发生变化,肿胀减小(下箭头),肾上腺病变也明显减小。


Discussion

TB is one of the mostancient multi-systemic, granulomatous infectious diseases recorded in humanhistory. However, its unusual presentations still elude physicians even in thisera of advanced medicine. Although EPTB has a broad spectrum of clinicalmanifestations, primary involvement of the adrenal gland and spine with longterm follow up has been very rarely reported. We herein report a rareoccurrence of adrenal insufficiency secondary to primary adrenal TB accompaniedby spinal involvement and its long term follow up after 3 years from onset ofthe diagnosis.

讨论

结核病是人类历史有记载以来最古老的多系统性、肉芽肿性传染病之一。然而,即使在医学得到快速发展的当代,其不寻常的表现仍困扰着医生。尽管肺外结核(EPTB)具有广泛的临床表现,但主要累及肾上腺和脊柱的结核,并进行长期随访的文献鲜有报道。本文报道了一例受原发性肾上腺和脊髓结核累及,继发肾上腺功能不全,并从诊断开始即进行长达3年随访的罕见病例。  


The patient had EPTB,both adrenal and spinal, resulting in adrenal insufficiency and lumbar backpain. For several months prior to the presentation, back pain had been presentand this may be evidence that hematogenous spread to the adrenal glandsoccurred from the lumbar vertebrae.

患者在肾上腺和脊柱均出现肺外结核,进而导致肾上腺功能不全和腰背疼痛。在出现这些表现前几个月,患者已经出现背部疼痛,这可能成为结核由腰椎通过血液传播扩散至肾上腺的证据。


Although adrenal TB isthe major cause of chronic primary adrenal insufficiency, especially indeveloping countries, common findings of Addison’s disease does not usuallyappear until more than 90% of adrenal tissue has been destroyed , explainingthe chronic nonspecific nature of the symptomatology in our patient andpositive anti-TB IgG which indicate the chronicity of infection.

肾上腺结核是慢性原发性肾上腺功能不全的主要诱因,尤其在发展中国家更常见,尽管如此,基本研究表明艾迪生氏病通常都在肾上腺遭到90%以上损伤时才表现出来。本文分析得出患者症状具有慢性非特异性的特点,其抗结核IgG呈阳性也表明患者是慢性感染。患者肾上腺受累表现以双侧肿块增大Yang等人曾报道称肾上腺双侧受累可作为原发性肾上腺肿瘤引发结核的最重要鉴别依据之一。


In our patient, theadrenal involvement was in the form of bilateral mass enlargement; Yang et alreported that bilateral involvement can be used as one of the most importantdiscriminators of TB from a primary tumor in the adrenal gland.

肾上腺结核通常会并发其他部位的结核,在本病例中,患者腰椎也受累出现了结核。


Adrenal TB usuallyoccurs together with the presence of extra- adrenal TB and in our case it isassociated with lumbar spine involvement. In skeletal TB, the upper lumbarspine is most commonly affected as seen in our case. The loose internalstructure of the disk allows the infection to disseminate more widely intoadditional spinal segments, resulting in the classic pattern of involvement of morethan one vertebral body together with the intervening disks, which exactlyoccurs in our case.

正如本病例中所显示的,在骨骼结核中,上腰椎是最常受到感染的部位。腰椎盘内部结构的疏松能促使结核感染范围更广,播散至其他脊柱节段,进而造成椎间盘受累的多脊椎节段受累的经典模式,在本病例中也出现了这些状况。  


Although adrenalcortex has considerable capacity of regeneration, Addison’s disease due totuberculosis is generally regarded as irreversible. Only a few patients withtuberculosis showed recovery of adrenal function . Very recently Anaforoğlu etal reported that stoppage of steroid therapy for patient with adrenalinsufficiency secondary to TB is associated with relapse of the symptoms asseen in our patient and in their 1 year follow up of the adrenal gland by MRIthey didn’t find any significant radiological changes. However, the improvedradiological findings in comparison with the old CT film in our patient is notassociated with restoration of the normal function of the adrenal glands;explaining the extension of steroid therapy in our patient.

尽管肾上腺皮质具有相当强大的再生能力,艾迪生氏病却因为是结核感染一般不可逆。仅少数结核病患者肾上腺功能得以恢复。Anaforoğlu等人最近报道称继发于结核的肾上腺功能不全的患者症状的复发与暂停类固醇药物治疗有关,本病例中即出现了此种状况。随后1年的随访中,患者肾上腺磁共振成像没有发现任何明显的X线变化。然而,患者改善后的X线片与之前的X线片相比,发现与肾上腺功能恢复程度不相关;这解释了患者需要扩大类固醇治疗的原因。


Conclusion

Physicians need to beaware of the widely different manifestations of EPTB which is a potential causeof adrenal insufficiency. Even with the improvement on the adrenal glandsradiological findings, steroid therapy may be needed to be continued for a longtime.
结论

肺外结核是肾上腺功能不全的潜在诱因,医生需知晓肺外结核(EPTB)的多种不同表现。即使肾上腺放射学研究已有进展,在很长一段时间内类固醇治疗仍是必须的。



Medicool医库软件 王露黔 赵美凤 编译

原文来自 Pan African Medical Journal

最后编辑百里小溪 最后编辑于 2016-07-11 15:13:26
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