lNTRODUCTlON

pneumonia (PJP) is more likely to occur in patients with immunodeficiency or longterm immunosuppressive therapy[1]. Early onset is insidious, progresses rapidly, and may even be lifethreatening. Approximately 1%-2% of patients with rheumatic diseases develop PJP, especially when glucocorticoids are combined with immunosuppressive therapy[2]. Some rheumatologists believe that patients with dermatomyositis have a higher risk of PJP when the same intensity of immunosuppression is used[3]. A study included 293 cases of PJP from 1990 to 2010, of which inflammatory diseases accounted for 15%[3]. The population with the highest risk (incidence ＞ 45/100000) included patients with dermatomyositis. In non-human immunodeficiency virus (HIV)-infected patients, the most important risk factor for PJP is the use of glucocorticoids[4]. A 12-year follow-up study from Norway included 297 non-HIV-infected patients with a first episode of PJP, 72.1% of whom had used glucocorticoids before diagnosis. The perioperative management of patients with dermatomyositis treated with long-term glucocorticoids needs to be carefully managed. The occurrence of PJP in the perioperative period is rare after kidney transplantation[5], but it has not been reported after arthroplasty.

No sooner had she reached her room than the Princess exclaimed, Now let us see what these fine plums can add to my beauty, and throwing off her hood52, she picked up a couple and ate them

CASE PRESENTATlON

Chief complaints

A 61-year-old woman was admitted to the hospital for "bilateral osteonecrosis of the femoral head (ONFH) for 1 year after long-term use of glucocorticoids".

History of present illness

The patient was diagnosed with bilateral ONFH (Figure 1A and B) one year prior because of the longterm use of glucocorticoids. When the patient was admitted to the hospital, the control of the primary disease was stable (Figure 2A). She underwent right total hip arthroplasty (Figure 1C and D) in the orthopaedic department. Hydrocortisone (50 mg) was administered through intravenous drip 1 d before, during, immediately after, and 1 d after the operation. The patient resumed 8 mg of oral methylprednisolone on the second postoperative day. On the fifth day after surgery, the patient suddenly developed dyspnea when walking, accompanied by chest tightness, palpitation, and blood in the sputum.

History of past illness

The patient had a history of anti-melanoma differentiation-associated gene 5 (MDA5)-positive dermatomyositis and interstitial pneumonia for 3 years. She had was administered 8 mg of methylprednisolone daily and 50 mg of cyclosporine every 12 h to control her condition.

I know how to obey orders, he said, and these are as plain as the nose on my face! So the princess was summoned, and, somewhat to their surprise, she seemed to take the news very calmly; next Nur Mahomed was informed, and he was greatly startled, but of course he could but be delighted at the great and unexpected honour which he thought the king had done him

Personal and family history

She was born in Shenyang and has lived there almost all her life. The patient’s living conditions were good, and she has no bad personal habits or customs. Her parents have both died.

I had only been there for about six months and was still in awe3 of the strength and power the love in a big family can generate. What they did that year was long-standing tradition for them, but I had never seen anything like it.

Physical examination

We are grateful to the family of the patient for their consent to publish this case report.

A few days after the crash, Sonali s brother Chris, concerned that Sonali might not understand what was really happening, asked her, Do you know where Daddy is?

Laboratory examinations

Considering severe pneumonia, the patient was transferred to the respiratory intensive care units for further treatment. Noninvasive mechanical ventilation and high-flow oxygen inhalation were administered, and SpOwas maintained at 98%-100%. She was administered 1.2 g of oral trimethoprimsulfamethoxazole (TMP-SMX) every 6 h (total of 5 d), 8 mg of methylprednisolone every day, and 50 mg of cyclosporine every 12 h. On the ninth day after surgery, the mask was replaced with oxygen inhalation. The results of arterial blood gas analysis showed the following:FiOof 0.31, pH 7.465, PaCOof 36.0 mmHg, PaOof 145 mmHg, HCOof 26.5 mmol/L, BE of 2.1 mmol/L, Lac of 0.8 mmol/L, SaOof 98.0%, and an oxygenation index of 467 mmHg. Ambroxol hydrochloride (60 mg) was administered intravenously every day for 3 d. On the 11th day after surgery, oxygen inhalation was changed to a nasal cannula. The WBC count and ALC were normal, the CRP level was 1.72 mg/dL, and the creatinine level was 99.6 μmol/L. The CT scan (Figure 2C) showed ground glass and mesh shadows in both lungs, which were significantly reduced compared with the onset. TMP-SMX was adjusted to 0.8 g every 6 h (total of 5 d), and cyclosporine was stopped.

On the 16day after surgery, the WBC, CRP, ALC, and creatinine levels were normal. The results of arterial blood gas analysis without oxygen inhalation showed the following:pH 7.438, PaCOof 33.1 mmHg, PaOof 74.5 mmHg, HCOof 23.4 mmol/L, BE of 1.5 mmol/L, Lac of 1.4 mmol/L, SaOof 95.6%, and an oxygenation index of 354 mmHg. The patient was discharged from the hospital and continued to take 8 mg of methylprednisolone every day and 0.8 g of TMP-SMX every 8 h. One month after surgery, cyclosporine was gradually increased to 50 mg every 12 h, and the administration of methylprednisolone (8 mg/day) and TMP-SMX (0.8 g/day) were maintained. There was no recurrence 6 mo after surgery. The CT scan (Figure 2D) showed a significant improvement compared to the images at the time of discharge.

Imaging examinations

The computed tomography (CT) scan (Figure 2B) showed diffuse grid shadows and ground glass shadows in both lungs, and left pleural effusion was distributed throughout the upper lobe of both lungs.

Further diagnostic work-up

FlNAL DlAGNOSlS

The patient was eventually diagnosed with PJP.

TREATMENT

Laboratory tests showed a high white blood counts (WBC) count of 16.14 × 10/L, a high absolute lymphocyte count (ALC) of 0.81 × 10/L, a high C-reactive protein (CRP) level of 7.54 mg/dL, a high erythrocyte sedimentation rate of 75 mm/h, and a normal creatinine level of 85.9 μmol/L. The results of arterial blood gas analysis showed the following:pH 7.49, PaCOof 32 mmHg, PaOof 47 mmHg, HCO- of 26 mmol/L, BE of 2.1 mmol/L, and Lac of 1.5 mmol/L.

OUTCOME AND FOLLOW-UP

For, she said, you see, Featherhead, I love you, and love makes everything right! But we cannot stay here, she added; what are we to do? The Prince thought they had better find their way to the Fairy of the Beech-Woods and put themselves once more under her protection, and they had hardly agreed upon this course when two little chariots wreathed with jasmine and honeysuckle suddenly appeared, and, stepping into them, they were whirled away to the Leafy Palace

DlSCUSSlON

This case posed a major challenge to the perioperative treatment and prevention of patients with autoimmune diseases and left important lessons for practice and research. First, the continued use of glucocorticoids in anti-MDA5-positive dermatomyositis patients may lead to immunosuppressive opportunistic infections. Second, surgery increases the additional conditions of immune stress and susceptibility to infection. Finally, the best management strategy for perioperative immunosuppressive therapy remains inconclusive due to a lack of evidence. Our treatment is consistent with the guidelines proposed by the American College of Rheumatology/American Association of Hip and Κnee Surgeons Guideline for the Perioperative Management of Antirheumatic Medication in Patients with Rheumatic Diseases Undergoing Elective Total Hip or Total Κnee Arthroplasty[6].

And the little hut turned facing her and stood still. Then smelling all around her, she cried: Foo! Foo! I smell a smell that is Russian. Who is here?

For non-HIV-infected patients with PJP, the first choice is TMP-SMX treatment for 21 d[18]. Non-HIVinfected immunocompromised patients treated for PJP usually have worse outcomes than HIV-infected patients. The mortality rate of PJP in HIV-infected patients is 10%-20%, while that in non-HIV-infected patients is 35%-50%[19]. There is no strong immunosuppressive therapy that can severely suppress the human immune system. Lowering the counts of lymphocytes and CD4 lymphocytes will increase opportunistic infections such as,and[19]. TMP-SMX treatment is recommended for the following high-risk populations to prevent the occurrence of PJP:Patients who have received ≥ 20 mg/d prednisone (or equivalent dose) for ≥ 1 mo and have other causes of immune function[18].

The management method of stress-dose glucocorticoids for autoimmune diseases in the perioperative period is mainly based on low-quality evidence and expert opinions. Existing research data are relatively limited, and the practice of clinicians is not the same. The use of glucocorticoids should be based on the possibility of hypothalamic-pituitary-adrenal axis (HPA) axis inhibition and the degree of surgical stress. Patients who use any dose of glucocorticoids for no more than 3 wk or ＜ 5 mg/d of prednisone (or equivalent doses) in the morning will not have HPA axis suppression and do not require additional glucocorticoids in the perioperative period[20]. For patients who are taking prednisone ＞ 20 mg/d (or equivalent doses) or are complicated with Cushing’s syndrome, glucocorticoids should be supplemented according to the degree of stress during the perioperative period[21]. For patients who use 5-20 mg/d of prednisone (or equivalent dose) ＞ 3 wk, an evaluation is recommended before surgery because of the substantial difference in HPA axis inhibition[20].

For patients with autoimmune diseases who require glucocorticoid supplementation during the perioperative period, it is necessary to use low-dose glucocorticoids to minimize other risks, such as infection. If the patient needs ＞ 10 mg/day of prednisone (or equivalent dose), it means that the disease has not been adequately controlled, and elective surgery should be postponed[22]. A propensity score matching study analyzed data from 9911 rheumatoid arthritis (RA) patients who underwent elective total knee or hip replacement surgery and found that glucocorticoids were dose-dependently associated with postoperative infection, hospitalization, and increased risk of artificial joint infection[23]. Compared with patients who did not receive glucocorticoid therapy within 90 d of knee or hip arthroplasty, patients who used ＞ 10 mg/day of prednisone (or equivalent doses) were expected to have a higher risk of hospitalization for infection (13.25 %6.78%)[23]. The cumulative incidence of periprosthetic joint infections is expected to be higher in one year (3.83%2.09%)[23]. Another populationbased study included data from 381 knee or hip replacement surgeries in 259 patients with RA. The risk of infection after replacement can increase by up to 20 times[24].

CONCLUSlON

Balancing the benefits and risks of glucocorticoids in the treatment of autoimmune diseases in the perioperative period remains a difficult question. Continued use of glucocorticoids in patients with anti-MDA5-positive dermatomyositis may lead to PJP. The use of glucocorticoids should be determined based on the possibility of HPA axis inhibition and the degree of surgical stress. For patients with autoimmune diseases who require glucocorticoid supplementation during the perioperative period, low-dose glucocorticoids should be used as much as possible to minimize the risk of PJP.

Polymerase chain reaction (PCR) testing of bronchoalveolar lavage fluid (BALF) was positive for.

The patient's temperature was 38.3 °C. She had tachypnea (respiratory rate:30 breaths/min) and tachycardia (heart rate:115 beats/min). Her blood pressure was 126/78 mmHg, and her blood oxygen saturation was 86%. She was conscious and able to answer questions correctly. Heart sounds and abdominal examinations were normal, and there was no increase in lower extremity edema and facial rash.

When they had got there, the West Wind asked him if he could tell her the way to the castle that lay east of the sun and west of the moon, for she was the girl who ought to marry the Prince who lived there

Hong M and Sun XW were responsible for the patient’s management and data collection and drafted the manuscript; Zhang ZY was responsible for the language review and drafted the manuscript; Wang WG performed the elemental analysis; Guo WS was responsible for analyzing the CT scan and editing the figures; Zhang QD was responsible for the interpretation of data and supervised this manuscript; all authors have read and approved the final manuscript.

The severe respiratory complications of dermatomyositis pose a considerable challenge for diagnosis and treatment, and the prognosis is poor[7]. Differential diagnoses include cardiac complications, respiratory muscle weakness, drug allergy, rapid progressive interstitial lung disease (RP-ILD), and severe opportunistic infections[8]. In many cases, it is difficult to make a definitive diagnosis. There are several explanations for acute respiratory disease in this case. First, for patients with PJP risk factors, pneumonia, and suggestive radiographic findings, PJP should be considered. Typical radiographic features include bilateral diffuse interstitial infiltration[9]. If the chest radiograph results are normal, a high-resolution CT scan may show extensive ground-glass opaque areas or cystic lesions[9]. Our diagnostic method involves microbiological identification of pathogenic microorganisms when conditions permit. PCR testing of the BALF or induced sputum is necessary. Compared with HIVinfected people, the number ofin non-HIV-infected people is significantly lower[10]. When respiratory samples cannot be obtained safely, treatment can be initiated based on the patient’s risk, clinical manifestations, and serum β-D-glucan testing[11]. Second, the progression of this pneumonia does not exclude RP-ILD. The risk of RP-ILD in anti-MDA5-positive patients is more than 20 times higher than that in anti-MDA5-negative patients[12]. Approximately 42%-100% of patients develop RP-ILD soon after the onset of disease and rapidly develop respiratory failure[13]. Despite active respiratory support and intensive immunosuppressive therapy, the effect is poor, and the prognosis is extremely poor, which is the main cause[14]. This patient had no exacerbation of the rash, and the serum ferritin level was not high, which is different from the RP-ILD symptoms caused by typical dermatomyositis. Finally, anti-MDA5-positive dermatomyositis patients appear to be predisposed to developing PJP from the outset, not just in relation to the immunosuppressive regimen they are receiving[15]. Li[16] reported that the 7 of 8 patients with dermatomyositis who developed PJP infection were anti-MDA5-positive. Aymonier[17] reported two anti-MDA5-positive dermatomyositis patients who developed RPILD due to PJP and eventually died after receiving immunosuppressive therapy.

National Natural Science Foundation of China, No. 81673776, and No. 82072494.

Then Little Two-eyes came out from under the cask quite happily, and the knight was astonished at her great beauty, and said, Little Two-eyes, I am sure you can break me off a twig from the tree

Informed written consent was obtained from the patient for the publication of this report and any accompanying images.

On my very last day in the orphanage5 I was being chased by the police. They told me that I was being sent off to the Florida School for Boys Reform School, at Marianna so I ran to get away from them. They chased me around the dining room building several times and finally I made a dash for the chain-link fence and tried to climb over in order to escape. I saw the deaf boy sitting there on his porch just looking at me as they pulled me down from the fence and handcuffed me. The boy, now about twelve jumped up and ran across San Diego Road, placed his fingers through the chain-link fence and just stood there looking at us.

The authors declare that they have no conflicts of interest.

The authors have read the CARE Checklist (2016). The manuscript was prepared and revised according to the CARE Checklist (2016).

This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BYNC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is noncommercial. See:https://creativecommons.org/Licenses/by-nc/4.0/

China

Mao Hong 0000-0001-8418-1950; Zi-Yu Zhang 0000-0003-3070-9910; Xiao-Wei Sun 0000-0003-1915-9161; Wei-Guo Wang 0000-0003-2953-2206; Qi-Dong Zhang 0000-0002-5890-0509; Wan-Shou Guo 0000-0003-4211-9117.

Fan JR

A

Fan JR