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46 Universal calcinosis in juvenile dermatomyositis: a case report

Abstract Background Dermatomyositis (DM) is an inflammatory myopathy that is often severe and affects children (juvenile DM) in 50% of cases. Subcutaneous calcinosis is seen in 30–70% of juvenile DM and often poses a problem in terms of treatment. We report a patient with diffuse subcutaneous calcinosis in juvenile DM. Observation The patient is K.S, a 17-year-old girl, with background history of congenital heart disease (atrial septal defect), which was corrected surgically in 2021. Since the age of 13, she has had DM for which she has received corticosteroid therapy at a dose of 2 mg/kg/day and improved clinically. The clinical examination reveals subcutaneous nodules in the arms, forearms, phalanx of the right index finger, inner thigh, knees and legs, associated with reduced range of movement of the right elbow (presence of calcifications) and limitation of internal rotation of the right hip. Laboratory investigations showed no inflammatory syndrome, the muscle enzymes and electromyogram were normal. Radiographs showed calcifications at the sites with subcutaneous nodules. Bone densitometry showed a decrease in bone mineral density (Z score= -3.4 standard deviation at the lumbar spine). The thoracic CT scan showed interstitial lung disease. The patient was managed with pamidronic acid for the diffuse calcinosis. She is also on diltiazem for Raynaud's phenomenon and hydroxychloroquine. Conclusion Subcutaneous calcinosis is diffuse in our patient. Treatment with bisphosphonates (pamidronic acid) is recommended.


Background
Pediatric onset Systemic lupus erythematosus (p-SLE) is multisystem autoimmune disease with disease onset before the 18th birthday. It is a highly complex disease with marked heterogeneity between patients, causing anything from mild to life-threatening disease. Objective To assess thrombomodulin (TM) and D-dimer levels in pediatric systemic lupus erythematosus patients and their correlation to disease activity.

Methods
This study included 40 children diagnosed with systemic lupus erythematosus. They were grouped according to the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score into group (A) [Mild activity] included 20 children with SLEDAI score less than or equal to 4, group (B) [Moderate-high activity] included 20 children with SLEDAI score >4. Also, 40 apparently healthy children were included as a control group. They were age and sex matched, with the children having SLE.

Results
The two studied groups had significantly higher TM level than controls, furthermore group B had significantly higher TM level than group A (p< 0.008 in all). The two studied groups had significantly higher frequency of abnormally high TM level than controls, furthermore group B significantly had higher frequency of abnormally high TM level than group A (p< 0.02 in all). SLE children had significantly higher D dimer level than controls, furthermore group B had significantly higher D dimer level than group A (p 0.006 in all). The two studied groups had significantly higher frequency of abnormally high D dimer level than controls, furthermore group B had significantly higher frequency of abnormally high D dimer level than group A (p 0.03 in all). TM had a significant positive correlation with SLEDAI score as r ¼ 0.342 and p ¼ 0.031. D dimer had a significant positive correlation with SLEDAI score as r ¼ 0.42 and p ¼ 0.007.

Conclusion
Thrombomodulin and D dimer are potentially valuable markers of the disease activity and thrombotic tendency in p SLE patients. Thus, regular evaluation may be useful for early detection of thrombotic complications. Background Systemic Lupus Erythematosus (SLE) is a chronic autoimmune disease that can involve any organ system with a wide range of disease manifestations, and can lead to significant morbidity and even mortality.

Objectives
To describe the epidemiology, common clinical features, complications, and management of SLE in children.

Methods
A single center retrospective descriptive study of 35 children with SLE.

Results
There were 27 (77%) girls and 8 (22.9%) boys. The mean age at diagnosis was 9 AE 2 years, with extremes of 3 and 15 years. Systemic involvement was in the form of protracted fever in 62.8%, nephrotic syndrome in 37%, nephritic syndrome in 40%, thrombotic microangiopathy in 8.6%, isolated proteinuria in 25.7%, acute kidney injury in 25.7%, malar rash in 48.6%, arthritis in 57%, neurolupus in 17%, serositis in 11.4%, and hemolytic anaemia in 28.6%. Coexisting autoimmune conditions were systemic sclerosis, antiphospholipid antibodies syndrome and Gujerat-Sjogren syndrome. Two cases had severe pancreatitis as a revealing pattern. Posterior uveitis was present in one case. Meanwhile, pulmonary involvement was seen in two cases (interstitial lung disease and recurrent pneumonia). Proliferative lupus nephritis was found in kidney biopsy in 48.6%. Four cases had end stage kidney disease and had hemodialysis sessions. Management consisted of hydroxychloroquine at 5 mg/kg/day and oral steroids at a starting dose of 2 mg/kg/day in all cases, while pulses of methylprednisolone were prescribed in 57%. Other immunomodulatory treatments used were IV cyclophosphamide in 34.4%, mycophenolate mofetil in 51.4%, azathioprine in 8.6%, tacrolimus in 5.7%, rituximab in 11.4%, and methotrexate in 2.8%. Evolution was marked by stabilization in 42.8% and recurrent flares in 28.6%. Meanwhile six cases were lost to follow-up and four cases died. The reasons for mortality were severe disease activity (seen in two cases), infection and macrophage activation syndrome (in one case each). Background Dermatomyositis (DM) is an inflammatory myopathy that is often severe and affects children (juvenile DM) in 50% of cases. Subcutaneous calcinosis is seen in 30-70% of juvenile DM and often poses a problem in terms of treatment. We report a patient with diffuse subcutaneous calcinosis in juvenile DM.

Observation
The patient is K.S, a 17-year-old girl, with background history of congenital heart disease (atrial septal defect), which was corrected surgically in 2021. Since the age of 13, she has had DM for which she has received corticosteroid therapy at a dose of 2 mg/kg/day and improved clinically. The clinical examination reveals subcutaneous nodules in the arms, forearms, phalanx of the right index finger, inner thigh, knees and legs, associated with reduced range of movement of the right elbow (presence of calcifications) and limitation of internal rotation of the right hip.
Laboratory investigations showed no inflammatory syndrome, the muscle enzymes and electromyogram were normal. Radiographs showed calcifications at the sites with subcutaneous nodules. Bone densitometry showed a decrease in bone mineral density (Z score¼ -3.4 standard deviation at the lumbar spine). The thoracic CT scan showed interstitial lung disease. The patient was managed with pamidronic acid for the diffuse calcinosis. She is also on diltiazem for Raynaud's phenomenon and hydroxychloroquine. Conclusion Subcutaneous calcinosis is diffuse in our patient. Treatment with bisphosphonates (pamidronic acid) is recommended. Background Childhood-onset systemic lupus erythematosus (c-SLE) is the prototype of a multisystemic, inflammatory, heterogeneous autoimmune condition, characterized by simultaneous or sequential organ involvement. Compared with the adult onset form, c-SLE is thought to have a worse prognosis.

Objectives
To review the epidemiological, and bio-clinical, characteristics of a c-SLE case series.

Methods
The files of patients diagnosed as c-SLE in the pediatrics department of Monastir, Tunisia from January 2004 to March 2022 were reviewed. Mean and standard-deviations were used to express normallydistributed variables, as verified by the Kolmogorov-Smirnov statistical test.

Results
Fourteen patients were collected. Female to male ratio was 6:1. Mean ages at lupus onset and diagnosis were 9.9 AE 1.4 years, [5-13.8 years] and 10.75 AE 2.3 years [6-14 years], respectively. Only two children had a family history of autoimmune disease. The initial admission was motivated primarily by skin and musculoskeletal manifestations, in 64.3% and 51.7% of cases, respectively. General signs (fever, asthenia) were observed in 35.7% of cases, hematological and gastrointestinal manifestations in 28.6% of cases each. In 3 cases, upper gastric endoscopy was performed prior to admission, in view of abdominal pain and vomiting. The physical examination noted various anomalies. Malar rash (50%) and discoid lupus (28.6%) were the most frequent cutaneous manifestations, while skin biopsy was performed in three cases, all favorable for lupus. The musculoskeletal manifestations comprised arthralgia (71.4%), arthritis and myositis (14.3% each). Hematological manifestations included thrombocytopenia and leukopenia in 4 cases each, as well as 3 cases of auto-immune hemolytic anaemia and splenomegaly. Renal manifestations were dominated by proteinuria in 7 cases, followed by hematuria in 6 cases, and 2 cases of hypertension (associated with renal failure in one case). The renal biopsy was performed in one case showed a stage-2 lupus nephritis. Pleural effusion was observed in 3 cases, 3 cases of pneumonia, 2 cases of pericarditis, one case of myo-pericarditis and one case of central nervous system (CNS) lupus. Relevant results of the laboratory workup are illustrated in the following The formal diagnosis of SLE was established according to the ACR-1997 criteria in 7 cases (50%), the SLICC-2012 in 4 cases (28.6%) and EULAR/ACR-2019 in 3 cases (21.4%). The c-SLE diagnosis was associated with coeliac disease and Hashimoto thyroiditis in one case each.
The therapeutic management was based on corticosteroids in 11 cases, followed by hydroxychloroquine in 3 cases, while cyclophosphamides and immunoglobulin were used for one case each. The outcomes were heterogeneous. Among 11 patients with sufficient follow-up, 6 cases of remission and 2 cases of relapse were noted. Major adverse events were not infrequent: one case each of cardiac tamponade, macrophage activation syndrome and severe CNS lupus were observed, all fatal.

Conclusion
Childhood-onset systemic lupus is a challenging disease, both to diagnose and to treat. The development of new criteria of higher specificity and sensitivity has greatly helped identify the incomplete types of lupus and allow for early stage diagnosis, and therefore prevent the serious complications of the disease. Background Juvenile dermatomyositis (JD) is a rare autoimmune disorder characterized by a non-infectious inflammatory state affecting of the muscles and skin associated with a vasculopathy which represents the essential of its physiopathogenesis.

Objectives
To review the clinical and biological diagnosis of DMJ as well as its evolution under treatment based on the cases of two patients at the Military Pediatrics Department of Tunis.

Observations
Case 1: This was a three-and-a-half-year-old child with a history of a simple febrile seizure, admitted for a skin rash and myalgia that had been evolving for eight months. Skin examination revealed an erythematous rash of the face and upper eyelids with erythematous papular lesions in front of the proximal interphalangeal joint recalling Gottron's papules. Muscle testing was in favor of a proximal deficiency and the electromyogram (EMG) revealed a diffuse myogenic process more pronounced on the proximal muscles without signs of myositic activity. Biology analysis did not reveal a biological inflammatory syndrome but rather increased muscle enzymes levels up to 3-and 4times normal values. On the immune status report, there was no evidence of DMJ-specific autoantibodies (ASM), but the anti-SRP antibodies associated with myositis were present. The diagnosis of DMJ was established in view of the association of typical cutaneous signs and 3 muscular signs (proximal deficit, elevation of muscle enzymes, myogenic tracing) and an evaluation of the activity of the disease according to a standardized scale by the Childhood Myositis Assessment Scale (CMAS) was required (which was at 7/52). Treatment with corticosteroids and methotrexate was started after a pretherapeutic evaluation and regular check-ups of the child showed a clear improvement of clinical and muscular signs (CMAS became at 41/52). Case 2: A 7-year-old boy, with no previous pathological history, admitted to our department to explore his gait disorder associated to erythematosquamous lesions. At the dermatological inspection, a localized erythema was noted on the upper eyelids, the nose, the cheeks and the chin area, as well as erythematosquamous lesions on the interphalangeal joints, metacarpophalangeal joints, elbows and knees (Gottron papules). The gait was waddling, the EMG was in favor of myogenic disorder and the muscular testing revealed a proximal muscular affection. The biology results showed an elevation of CPK and LDH to 1.5 times the normal values and a sedimentation rate at 40 mm/h. The antibodies determination showed the existence of anti-NXP2 antibodies specific to myositis. The progression under treatment (corticosteroids, methotrexate and adjuvant treatment) was favorable with a CMAS score of 39 (CMAS was 11 when admitted). The persistence of squamous lesions recalling calcinosis on the dorsal surfaces of the forearms was well correlated with the presence of anti-NXP2 antibodies.

Conclusion
The positive diagnosis of DMJ in a child must be based on clinical and biological arguments. The immunological characterization of DMJ should be an important element in the disease management because it can explain the lesions associated with certain myositis specific antibodies as well as their evolution, which implies a particular followup depending on the detected antibodies (risk of cancer in anti-NXP2 AC positive patients)