MAEDICA – a Journal of Clinical Medicine Mædica - a Journal of Clinical Medicine 2020; 15(3): 394-400 https://doi.org/10.26574/maedica.2020.15.3.394 Case reports Respiratory Failure in a Rare Case of Juvenile – Systemic Nicoleta Aurelia POPESCUa, Dana MANEAa, b, Georgiana CAPITANESCUa, Eliza CINTEZAc, d, Marcela Daniela IONESCUa, c, Mihaela BALGRADEANa, c aPediatric Department, “M.S. Curie” Children’s Emergency Hospital, Bucharest, Romania bAnaesthesiology and Intensive Care Therapy Unit, “M.S. Curie” Children’s Emergency Hospital, Bucharest, Romania c”Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania dPediatric Cardiology Department, “M.S. Curie” Children’s Emergency Hospital, Bucharest, Romania

BACKGROUND Juvenile dermatomyositis (JDM) is one of the pediatric systemic connective tissue disorders, consisting of an idiopathic inflammatory , affecting primarily skin and muscle, representing approximately 85% of cases in this group. A significant source of morbidity is the occurrence of overlap characteristics with other connective tissue disorders, including systemic sclerosis (SSc). Overlap JDM/SSc syndrome is rare in children, with only a few reported cases. The diagnosis is often challenging, presence of anti-PM/Scl antibodies playing a pivotal role. Although SSc/JDM overlap syndrome has less frequent visceral involvement, pulmonary dysfunction may occur. The respiratory function evaluation using overnight cardiorespiratory polygraphy may reveal important alveolar hypoventilation with impact on therapeutic approach. Non-invasive ventilation may be indicated to potentiate medical treatment. In the acute phase, non-invasive ventilation is a life-saving therapeutic option until the maximum efficiency of drug treatment is reached. In the case of a complex respiratory pathology, associating elements of nocturnal alveolar hypoventilation specific to neuromuscular disease, with that of chronic interstitial lung disease, the evaluation of respiratory sleep disorders should be considered, sometimes requiring home nocturnal non- invasive ventilatory support. We present the case of a 15-year-old girl who was admitted to our clinic with a history of high fever, productive cough and severe dyspnea. Detailed anamnesis revealed that the patient accused one- year history of proximal of the lower limbs, with functional limitations, weight loss, dysphonia, swallowing difficulties and dyspnea at minimal efforts. Following the physical examination, laboratory and imagistic investigations were all suggestive for an . Anti-PM/Scl antibodies were positive, confirming the diagnosis of a severe form of JDM/SSc overlap syndrome, with minimal cutaneous changes, significant muscle involvement and respiratory distress. Complex therapy using antimicrobial agents, steroid pulse therapy, immunosuppressive agents, non-invasive ventilation,

Address for correspondence: Marcela Daniela Ionescu Email: [email protected]

Article received on the 16th of September 2020 and accepted for publication on the 22nd of September 2020

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oxygen supplementation and physiotherapy was started, with significant status improvement. However, pulmonary function tests maintained severe restrictive aspect and nocturnal cardio-pulmonary polygraphy revealed residual pulmonary failure with important nocturnal alveolar hypoventilation. Nocturnal non-invasive ventilation was continued at home, along with medical treatment. Her disease was clinically well controlled, immunosuppressive therapy was decreased and interruption of ventilatory support was possible at six months after the diagnosis. Keywords: dermatomyositis-systemic sclerosis overlap, child, pulmonary dysfunction, overnight cardiorespiratory monitoring, non-invasive ventilation.

INTRODUCTION sclerodactyly and may progress to associate other SSc features. However, during the disease, at uvenile dermatomyositis (JDM) is one of the some periods, symptoms of scleroderma or der- systemic connective tissue disorders that af- matomyositis are prevalent, variously pronounced fect children, representing approximately in individual cases. Cutaneous changes of both 85% of cases in this group. The reported an- SSc and JDM develop within the first year of the nual incidence ranges from two to four cases disease. The JDM/SSc overlap differs from syste­ Jper one million children, with girls being affected mic scleroderma by the absence of digital pits and more often than boys. It consists of an idiopathic ulcer, acrolysis and of the fingers. inflammatory myopathy, affecting primarily skin Some sclerodermoid features of the are com- and muscle. Despite treatment improvements, it mon. Exceptionally, the onset can be sudden, is still associated with significant mortality and with fever, myalgia, Raynaud’s phenomenon. morbidity. An important source of considerable Muscle involvement can be insidious. is morbidity is the occurrence of overlap characte­ clinically indistinguishable from primary JDM. It is ristics with other connective tissue disorders, in- proximal, symmetric, causing function limitations, cluding systemic sclerosis (SSc), rheumatoid ar- such as frequent falls, difficulty in climbing stairs, thritis, Sjogren’s syndrome and systemic washing, feeding themselves. The muscle and cu- erythematosus (1, 2). Overlap JDM/SSc syndrome taneous signs of JDM are usually transient. Visce­ is rare in children, with only a few cases been re- ral involvement is often mild than in adults (5). ported. The diagnosis is often challenging. Al- Laboratory findings are supportive of the diagno- though it is a rare disease in children, the presen- sis: elevated levels of muscle enzymes, presence tation appears similar to adults, except for the of autoantibodies, elevated serum and urine myo- visceral involvement. In patients with unusual globin. The presence of anti-PM/Scl antibodies clinical features, an unusual clinical course or has a key role in confirming the diagnosis (3). those with scleroderma overlap features, testing Magnetic resonance imaging (MRI) of skeletal myositis-specific antibodies is useful. Usually, the muscles is helpful for detecting areas of muscle initial characteristic presentation in pediatric pa- . Electrophysiologic abnormalities tients is JDM (3). on electromyography (EMG) and muscle biopsy Juvenile dermatomyositis is diagnosed using cli­ are invasive diagnostic procedures that are re- nical, laboratory and microscopic methods. In 2017, served for cases in which other investigations are the European League Against Rheumatism/Ame­­ ­­ inconclusive (13). rican College of (EULAR/ACR) de- Although SSc/JDM overlap syndrome in chil- veloped revised the classification and diagnostic dren has less frequent visceral involvement than criteria for adult and juvenile inflammatory myopa- adults, pulmonary dysfunction may occur. Respi- thies, including variables related to muscle weak- ratory muscle weakness, affecting inspiratory, ex- ness, skin manifestations, laboratory findings and piratory and upper airway muscles, can cause in- other clinical manifestations. Muscle biopsies are sufficient ventilation, ineffective cough, nocturnal infrequently performed in children (4). hypoventilation, along with bulbar dysfunction. The occurrence of overlap features with other Impairment of both upper airway muscles and systemic connective tissue disorder creates diffi- upper esophagus induces difficulty swallowing, culties in diagnosis. JDM/SSc overlap presents tracheal aspiration or food reflux into the naso- with facial skin changes, Raynaud’s phenomenon, pharynx, dysarthria, nasal voice, weak mastica-

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tion, abnormal secretion clearance and protru­ motor vehicles and climbing stairs. Later, she no- ding tongue (1, 6). These perturbations, along with ticed the progressive association of voice changes decreased accessory respiratory muscle activity, – nasal voice, dysphonia, swallowing difficulties for may produce upper airway obstruction during in- liquids or solids, reflux of food into the nasopha­ spiration and exacerbate hypoventilation, espe- rynx, dyspnea at minimal efforts, involuntary weight cially during nocturnal sleep. An ineffective cough loss (8 kg through the last six months) and accen­ may be associated and the risk of aspiration, se- tuated proximal motor deficit. The antenatal, natal cretion retention, pneumonia and respiratory fai­ and postnatal histories were insignificant. There lure is very high (6). Although pulmonary manifes- was no family history of any neurological disease. tations are much less common in children than Physical examination upon presentation re- adults, interstitial lung disease (ILD) may occur. vealed an underweight patient, with a poor gene­ The pulmonary pathology is mostly extrapolated ral condition, inexpressive mimic, stretch marks in from adults because 30% to 50% of adults with the lower limbs, without other cutaneous sugges- dermatomyositis have ILD, whereas the pulmo- tive elements, apparent normal osteoarticular sys- nary expression in JDM/SSc or JDM is extremely tem, but with severe proximal muscle weakness rare in children (7). The diagnosis of ILD in pa- and fatigability to minimal efforts. Respiratory rate tients with known JDM can usually be established was 30 per minute and oxygen saturation was based on the clinical presentation, imaging stu­ 90% in room air. Decreased breath sounds in the dies and pulmonary function tests. The most com- lower right lung field, bilateral crackles and rhon- mon findings on chest radiography are diffuse re- chi were evident on pulmonary auscultation. In- ticular and nodular opacities. High-resolution tercostal and subcostal retractions, along with ab- computed tomography (HRCT) is preferred and dominal paradox breathing movement and typical changes include patchy ground-glass ineffective productive cough were also noticed. opacification, basilar consolidation, honeycom­ She was tachycardic, with rhythmic cardiac bing and septal thickening. Pulmonary function sounds and normal blood pressure. Neurological tests demonstrate a restrictive pattern, with de- examination revealed normal sensorium. Assess- creased lung volumes and diminished diffusing ment of cranial nerves showed normal oculo- capacity. Bronchoalveolar lavage or even lung bi- motricity, inexpressive facies with bilateral facial opsy may be indicated (1, 7). weakness (cannot whistle), absence of palatine The respiratory function evaluation using over- reflex, lateral atrophy and fasciculations of the night cardiorespiratory polygraph or even poly- tongue, dysarthria and nasal voice. She accused somnography may reveal important alveolar hy- difficulties in swallowing solids and liquids. The poventilation with impact on therapeutic muscle strengths of neck flexors and all, upper approach. In addition to medical therapeutic and lower, extremities were decreased at the measures, non-invasive ventilatory support may proximal level. The deep tendon reflexes were be indicated to control nocturnal alveolar hy- hyperactive in all extremities. Babinski’s and poventilation and other nocturnal respiratory dis- Gower’s signs were present. An otolaryngology orders, diurnal respiratory failure and to poten­ examination confirmed the velopalatine paralysis. tiate the medical treatment, inclusive the A nasopharyngeal endoscopy was also performed, treatment of acute respiratory (8). q with normal anatomic structures in this area. Laboratory investigations revealed normal CASE REPORT complete blood count, positive inflammatory tests (erythrocyte sedimentation rate 60 mm/h, C-reac- previously apparent healthy 15-years-old girl tive protein level 140 mg/dL), elevated creatine Awas admitted to our clinic with a history of kinase (CK), alanine aminotransferase (ALT), as- productive cough and high fever, lasting for eight partate aminotransferase (AST) and lactate dehy- days, progressive worsening despite symptomatic drogenase (LDH) levels (320 U/L, 50 U/L, 72 U/L treatment given by her general physician. To a and 502 U/L, respectively). Troponin T level was more detailed anamnesis, we found out that the also highly elevated (974 ng/dL), with no electro- patient accused one-year history of the lower cardiography or echocardiography abnormalities. limbs’ proximal muscle weakness, with functional Arterial blood gas analysis was normal. The chest limitations, such as difficulty getting into or out of radiograph showed a heterogeneous opacity in

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the right lower pulmonary lobe and an atelectatic nuclear and anti dsDNA antibodies results were band at this level, with diaphragm traction. Pul- early available, showing normal levels. monary functional testing could not be performed Despite initial complex medical treatment and because of poor status. respiratory nursing, the patient’s clinic and para- Firstly, we presumed the diagnosis of right infe- clinical course was undulating, with sudden dete- rior lobar pneumonia with respiratory failure, cen- rioration on 14th day of hospitalization, with an tral and peripheral motor neuron syndrome. We altered general condition, persistent hyperpyre­ started medical treatment with broad-spectrum xia, severe respiratory distress, bulbar paralysis antibiotics (Ceftriaxone, Vancomycin), bronchodi- and inability to perform antigravitational move- lator agents, respiratory nursing and oxygen the­ ments. Laboratory tests showed leukocytosis, rapy, with clinical, biological and radiological im- neutrophilia, positive inflammatory markers, per- provement. However, bulbar dysfunction and sistently elevated muscle enzymes and mild mixed muscle weakness persisted, with minimal allevia- acidosis. Chest radiography revealed large opacity tion. with air bronchogram occupying the inferior left In order to evaluate muscle weakness, electro- lobe, suggestive for pneumonia, associated with myography was performed, with inconclusive re- pleural effusion in the left lower lung, confirmed sults, suggestive for neurogenic abnormalities by thoracic ultrasonography. rather than myogenic lesions. Considering the pa- A neurologic reassessment was done, and a tient presenting symptoms of both central and pe- new electromyography was performed, revealing ripheral motor neuron syndromes associated with myogenic abnormalities. Muscle biopsy could not bulbar dysfunction and elevated levels of muscle be effectuated. Reevaluation of the case was im- enzymes, with a non-specific electromyography posed. Anamnestic data, consisting of insidious, aspect, further investigations were required. Mag- symmetrical, proximal muscle weakness for one netic resonance imaging (MRI) of the brain with year, swallowing and phonation disorder and im- entire spine screening was normal, eliminating the portant weight loss, along with physical exam, se- possibility of cerebral or medullary lesions (tumor, rum elevated muscle enzymes and EMG aspect, malformation, hemorrhage) and demyelinating or were all suggestive for an inflammatory myopathy. degenerative disease at this level. Myasthenia gra- Cutaneous characteristics were minimal, but we vis was excluded by the physical examination (ab- considered all clinical and paraclinical features sence of circadian variation of muscle weakness, suggestive for JDM. Nailfold capillaroscopy de­ absence of palpebral ptosis), along with electro- monstrated a lower density of capillaries, typical myography aspect, elevated muscle enzymes and cutaneous changes for JDM. Immunologic scree­ negative anti-acetylcholine receptor antibodies. ning autoantibodies panel for myositis performed Paraneoplastic involvement was taken into discus- as a part of the initial diagnostic workup, but with sion, with no evidence of tumors on chest radio­ late available results (one day after clinical dete­ graphy and abdominal ultrasound and a normal rioration), showed positive anti-PM/Scl 100 and level of alpha-fetoprotein level. Different forms of anti-PM/SCL 75 antibodies, so that the diagnosis progressive bulbar palsy have been also consi­ of JDM with scleroderma overlap syndrome was dered. In order to exclude Fazio Londe disease, sustained. Intravenous pulsed methylpredniso- plasma riboflavin levels and acylcarnitine levels lone was commenced, with significant improve- were performed, with normal values. Absence of ment of clinical status, in the first 24 hours, consti- sensorineural hearing loss eliminated hereditary tuting another argument for systemic inflammatory­ motor neuropathy type I. Slowly progressing re- disease diagnosis. duction of muscle strength, with prevailing ex- In order to assess the proper therapeutic plan, pression at girdle level, respiratory difficulties, or- we asked for an immunologic evaluation (at “Alfred thopnea, gradual bodyweight loss, swallowing Rusescu” Institute for Mother and Child), confir­ difficulties, elevated CK levels, were highly su­ ming the diagnosis of a severe form of JDM/SSc ggestive for late-onset Pompe disease, so that the overlap syndrome, with minimal cutaneous chan­ activity of alpha glucosidase was tested, being ges and important muscle involvement. In addition within its reference range; thus, there was no indi- to steroids, we started weekly . cation of Pompe disease. Screening for autoim- To clarify the extend of lung damage, HRCT mune illness was also performed, but only anti- was performed, revealing focal consolidations

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FIGURE 1. Thoracic HRCT – focal consolidations, micronodules, septal thickening and patchy ground glass opacities

with air bronchogram in the basal segment of the analysis showed a pattern of hypoxic-hypercapnic lower lobe of the left lung and in the medium lobe respiratory failure. of the right lung, with interstitial peribronchial As a result of severe respiratory distress, with changes: micronodules, septal thickening and mixt respiratory failure and ineffective cough, we patchy ground-glass opacities (Figure 1). considered it necessary to initiate continuous Pulmonary function tests were used to assess non-invasive ventilation along with oxygen thera- the severity of respiratory impairment. They de­ py, respiratory nursing and medical treatment. We monstrated the persistence of a restrictive pattern opted for bilevel positive airway pressure support, (low forced vital capacity – 21% of predicted, low with back up rate, associated with oxygen thera- total lung capacity – 52% of predicted) and dimi­ py, using a standard nasal mask, with an important nished alveolo-capillary diffusing capacity, taking improvement of the respiratory function. into consideration difficult examination because After three weeks of complex therapy using of tachypnea, respiratory muscle weakness and antimicrobial agents associated with steroids low pulmonary volumes. The arterial blood gas pulsed therapy, immunosuppressive therapy,

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DISCUSSIONS

verlap syndromes of JDM/SSc are rare and Ohave primarily been described in adults. In children, only few cases have been described. We reported the case of a young girl who presented an overlap syndrome of dermatomyositis and scleroderma, with positive anti-PM/Scl 100 and anti-PM/Scl 75 antibodies, highlighting the com- plex mechanism of pulmonary complications as a FIGURE 2. Overnight cardio-pulmonary monitoring test – result of systemic connective tissue disorder. nocturnal alveolar hypoventilation The main characteristics of JDM are symmetri- cal, proximal muscle weakness and chronic in- flammation of the skin. The diagnosis is suggested non-invasive ventilation and physiotherapy, the by the clinical features and confirmed through patient status was alleviated, with significant im- laboratory and microscopic methods. In our pa- provement of muscle force, reducing respiratory tient, the diagnosis was difficult to establish fol- distress with no need for oxygen therapy and pro- lowing a clinical examination dominated by mus- gressive weaning of diurnal non-invasive ventila- cle weakness, with minimal cutaneous changes. tion, improvement on respiratory clearance, ame- Her initial biological evaluation showed elevated lioration of swallowing and phonation. The patient muscle enzymes, positive inflammatory tests, neg- regained mobility and could maintain orthostatic ative antinuclear antibodies and anti-dsDNA anti- position and walk for short distances. The level of bodies. Electromyography, an invasive test used to muscle enzymes and blood gases normalized and distinguish muscle weakness caused by muscle serum inflammatory markers became negative. Re- denervation from that caused by inflammatory peated chest X-ray showed a regression of focal myopathy, may be falsely negative in some pa- infiltrations and pleural effusion. tients, as in our case, making the diagnosis more However, pulmonary function tests main- difficult. We could not perform a muscle biopsy tained severe restrictive aspect. As interstitial lung because of her severe pulmonary status. The pre­ disease with pulmonary fibrosis may develop se­ sence of anti-PM/Scl antibodies had a key role in condary to systemic connective disorders (derma- confirming the diagnosis of JDM/SSc overlap syn- tomyositis and scleroderma), and considering the drome. respiratory muscle weakness, we performed noc- Although JDM/SSc overlap syndrome has less turnal cardio-pulmonary polygraphy in order to frequent visceral involvement, pulmonary dys- evaluate the possibility of residual sleep-related function may occur. The extend of lung involve- breathing disorder. We revealed the persistence ment in patients with dermatomyositis may de- of nocturnal alveolar hypoventilation, with hyper- velop secondary to , aspiration capnia and hypoxemia (oxygen saturation < 90% pneumonia, reflux, interstitial lung disorder or res­ in room air for 63 percent of sleep time) (Figure 2). piratory muscle weakness. The decreased strength However, diurnal oxygen saturation was normal. of respiratory muscles induces inadequate ventila- We decided to continue nocturnal non-invasive tion, causing tidal volume to decrease. To main- ventilation at home, along with medical treat- tain a good tidal volume, patients use accessory ment. We scheduled a close follow-up, keeping in inspiratory muscles and increase respiratory fre- touch with the immunology department too. The quency. When the increase of respiratory rate be- subsequent evolution was favorable. Her disease comes insufficient to maintain alveolar ventilation, was clinically well controlled, and her immuno- hypercapnia develops. Inadequate venti­lation­ suppressive therapy was decreased. The pulmo- and low tidal volumes induce atelectasis, and nary function was also improved, with alveolar right to left pulmonary shunt develops, producing hypoventilation resolution on repeated nocturnal hypoxemia that accompanies insufficient ventila- cardiorespiratory monitoring polygraphy, so that tion. Impairment of upper airway muscles and the interruption of ventilatory support was permit- upper esophagus creates bulbar dysfunction, in- ted at six months after the diagnosis. q cluding difficulty in swallowing, tracheal aspira-

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A Rare Case of Respiratory Failure tion or food reflux into the upper airway tract. In ment in a young girl presenting severe respiratory our patient, the clinical picture was dominated by distress. This condition is a rare entity in child- respiratory symptoms and muscle disorders. Pul- hood and adolescence. Only a few cases have monary infection developed secondary­ to respira- been reported so far. It is associated with features tory muscle severe weakness, and the pleural ef- of SSc and dermatomyositis. Muscle weakness fusion probably secondary to infection rather than and severe respiratory illness dominated the di­ rheumatologic disorder. The progression of respi- ratory complications to acute respiratory failure, sease course, requiring ventilation support. We persistent with medical and supportive measures, highlighted the key involvement of anti-PM/Scl required a non-invasive ventilatory treatment. antibodies in establishing the diagnosis. Also, the Interstitial lung disease can occur, although it is case illustrates the reversibility of muscle weak- less common in children with JDM/SSc overlap ness in severe JDM/SSc overlap syndrome and syndrome. During the acute illness, our patient shows that active management, including non-in- could not undergo pulmonary function testing. vasive ventilation and treatment of chest infec- On recovery, she presented a persistent restrictive tions, are essential in-patient boarding. The im- pattern on repeated pulmonary functional tests, portance of nocturnal cardio-pulmonary polygraphy with low alveolo-capillary diffusing capacity and rare patchy ground-glass opacification on HRCT in the diagnosis of sleep-related breathing disor- examination. Also, nocturnal cardio-pulmonary der is sustained. In the presence of a multisyste­ polygraphy showed persistence of alveolar hy- mic pathology with severe neuromuscular and poventilation and hypoxemia in the complex con- respiratory impairment, association with noctur- text of severe muscle and pulmonary dysfunction nal hypoventilation and hypoxemia should be within the systemic inflammatory disorder. Home considered and specific tests (eg, nocturnal car- nocturnal non-invasive ventilation was continued diopulmonary monitoring test) should be proceed and closely follow-up was scheduled. Supportive in a time of clinical stability. Nocturnal non-inva- management of respiratory muscle weakness, in- sive ventilation is an important therapeutic solu- cluding ventilatory support, along with medical therapy, provided symptomatic relief and im- tion in this type of pathology. Once the resolution proved the quality of life. q of neuromuscular and pulmonary dysfunction is realized, withdrawal from nocturnal ventilatory CONCLUSIONS support is possible. q

e illustrated a rare case of JDM/SSc overlap Conflicts of interest: none declared. Wsyndrome with important visceral involve- Financial support: none declared.

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