进行性骨化性肌炎

进行性骨化性肌炎是以先天性拇趾畸形和肌健、韧带、筋膜以及骨胳肌进行性异位骨化为特征。开始为颈后、胸背部红、肿、疼痛、压痛、筋膜下结节，继而从近侧向远侧，从中轴向周围，从背侧向腹侧发展。有的结节自行缩小，有的则形成成熟的异位骨。由于继发关节外的僵直，形成严重的伤残。发病年龄在１０岁以内。根据临床特征和Ｘ线表现可以诊断。不宜活组织检查，以免促使病情恶化。     

Myositis (Fasciitis) Ossificans in an Infant

Myositis (fasciitis) ossificans developed following minor trauma to the soft tissues of the posterior aspect of the right knee of a 5-month-old female infant. The clinical and pathological features of myositis ossificans are reviewed and the differential diagnosis of soft tissue ossification in early childhood is discussed.     

Myositis ossificans in the soft tissues of the hand

Summary Myositis ossiticans is a benign sort tissue tumor of unknown etiology characterized by the rapid development of swelling and pain. The occurrence of this lesion in the hand is uncommon. Initially, the roentgenograms are normal, but progressively a bony mass can be visualized in the soft tissues. The lesion is often mistaken for infection or malignancy. The definitive diagnosis is confirmed by biopsy which shows a typical zone phenomenon with three well differentiated areas of pathologic findings. A case is presented to illustrate the classic clinical evolution, radiologic findings and treatment of myositis ossificans affecting the soft tissues of the hand. The surgical treatment obtained an almost complete remission of symptoms and pathologic signs.     

Myositis Ossificans Traumatica Causing Ankylosis of the Elbow

Myositis ossificans traumatica is an unusual complication following a muscle contusion injury. A significantly large myositic mass causing ankylosis of the elbow is even rarer. We report a 13-year-old boy who presented with a 14-month history of a fixed elbow with no movement and a palpable bony mass in the anterior aspect of the elbow. He had sustained significant trauma to the affected limb 1 month prior to onset of symptoms, which was managed by native massage and bandaging for 4 weeks. The clinicoradiological diagnosis was suggestive of myositis ossificans, and the myositic mass was completely excised. Histopathology revealed lamellar bone. The 2-year follow-up showed full function of the affected limb and no signs of recurrence. We report this case of clinical interest due to the unusually large myositic mass.     

Functional Outcome in a Patient With an Acute Quadriparesis Secondary to Systemic Sclerosis: A Case Report

Chernev I, Gustafson K, Medina-Bravo A. Functional outcome in a patient with an acute quadriparesis secondary to systemic sclerosis: a case report.Scleroderma or systemic sclerosis (SSc) is a relatively uncommon disease. Although well-known for many years, research on appropriate physical therapy during all stages of myositis due to scleroderma is limited. We report the functional outcome in a patient with an acute quadriparesis secondary to diffuse SSc associated with extensive myositis. This 35-year-old black woman progressed to almost complete functional recovery in the course of 16 days of acute rehabilitation with combined physical therapy including resistive exercises. This case strongly suggests that a patient with diffuse SSc and associated myositis can undergo aggressive physical therapy in a monitored environment with good functional improvement and no worsening of the myositis.     

Possible future avenues for myositis therapeutics: DM,IMNM and IBM

Idiopathic inflammatory myopathies (IIMs) represent a heterogeneous group of systemic autoimmune diseases characterized by immune-mediated muscle injury. As insights into pathogenesis of IIM evolve, novel therapeutic strategies have become available to optimize outcomes. Herein, we summarize novel and emerging strategies in the management of dermatomyositis (DM), immunemediated necrotizing myopathy (IMNM), and inclusion body myositis (IBM).     

Pregnancy in myositis and scleroderma

Myositis and scleroderma are both rare autoimmune diseases with female predominance and often occur before and during reproductive years. The rarity of diseases explains the low frequency of concurrent disease and pregnancy. Like other autoimmune diseases, myositis and scleroderma may be more active during pregnancy as well. To date, many patients with myositis and scleroderma can have favorable pregnancy outcomes with careful management. This chapter provides a current overview of pregnancy outcomes in myositis and scleroderma. A major theme that appears to have emerged across these inflammatory diseases is that active maternal disease during pregnancy is associated with adverse pregnancy outcomes, and thus, personalized management is necessary depending on the disease state and comorbidities.     

The natural history of fibrodysplasia ossificans progressiva: A prospective,global 36-month study

PurposeWe report the first prospective, international, natural history study of the ultra-rare genetic disorder fibrodysplasia ossificans progressiva (FOP). FOP is characterized by painful, recurrent flare-ups, and disabling, cumulative heterotopic ossification (HO) in soft tissues.MethodsIndividuals aged ≤65 years with classical FOP (ACVR1^R206H variant) were assessed at baseline and over 36 months.ResultsIn total, 114 individuals participated; 33 completed the study (mean follow up: 26.8 months). Median age was 15.0 (range: 4-56) years; 54.4% were male. During the study, 82 (71.9%) individuals reported 229 flare-ups (upper back: 17.9%, hip: 14.8%, shoulder: 10.9%). After 84 days, 14 of 52 (26.9%) imaged flare-ups had new HO at the flare-up site (mean new HO volume: 28.8 × 10³ mm³). Mean baseline low-dose whole-body computed tomography (excluding head) HO volume was 314.4 × 10³ mm³; lowest at 2 to <8 years (68.8 × 10³ mm³) and increasing by age (25-65 years: 575.2 × 10³ mm³). The mean annualized volume of new HO was 23.6 × 10³ mm³/year; highest at 8 to <15 and 15 to <25 years (21.9 × 10³ and 41.5 × 10³ mm³/year, respectively) and lowest at 25 to 65 years (4.6 × 10³ mm³/year).ConclusionResults from individuals receiving standard care for up to 3 years in this natural history study show the debilitating effect and progressive nature of FOP cross-sectionally and longitudinally, with greatest progression during childhood and early adulthood.