Pneumomediastinum in interstitial lung disease associated with dermatomyositis and polymyositis

Objective

Spontaneous pneumomediastinum is a rare complication of dermatomyositis (DM) and polymyositis (PM). The aim of this study was to characterize this complication and determine its prognostic factors.

Methods

We retrospectively collected a multicenter series of PM/DM cases complicated by pneumomediastinum. We analyzed all published cases and combined those that were exploitable with ours for an investigation of the factors associated with poor survival.

Results

We collected 11 PM/DM cases complicated by interstitial lung disease and pneumomediastinum. Five of the 9 DM patients had clinically amyopathic DM without muscle weakness and high serum creatine kinase levels. The outcome was favorable in 7 of these patients and 6 had no sequelae. In total, ～25% of our patients of the 21 analyzable cases studied died within 1 month. With a median followup of 240 days, the cumulative estimated Kaplan‐Meier survival rate was 64% at 1 year and 55% at 2 years. Poor survival was associated with absence of muscle weakness (P = 0.02), initial low vital capacity (P = 0.006), and initial low carbon monoxide diffusion capacity (P = 0.04).

Conclusion

In this first large series of patients with connective tissue disease complicated by pneumomediastinum to be reported, most patients had DM and half amyopathic DM, as in previous reports. Pneumomediastinum may occur before DM diagnosis and may thus reveal DM with minimal or no muscle involvement. Death was associated with an absence of muscle weakness and severe pulmonary involvement before the onset of pneumomediastinum. Corticosteroids and immunosuppressive therapy can result in complete recovery, as in half our cases.     

Severe diffuse interstitial pneumonia due to Mycoplasma pneumoniae in a patient with respiratory insufficiency

We report a 25-year-old man presenting with high fever, dyspnoea and somnolence. The presence of severe diffuse interstitial pneumonia with extrapulmonary symptoms, such as myositis and subclinical haemolysis, strongly suggested an infection by Mycoplasma pneumoniae. This diagnosis was supported by high titres of cold agglutinins and a positive Coombs test, and directly confirmed by specific IgM serological tests. After initiation of the appropriate antimicrobial treatment mechanical ventilation could be avoided and the patient showed a slow but complete clinical recovery. This diagnosis should be considered in any febrile patient with hypoxaemia and diffuse interstitial pneumonia, and rapid initiation of appropriate antibiotic treatment seems to be crucial for a favourable outcome.     

An autopsy case of idiopathic interstitial pneumonia with diffuse alveolar hemorrhage due to acute exacerbation]

There has hitherto been no report describing idiopathic interstitial pneumonia associated with diffuse alveolar hemorrhage, but we herein report one such rare case. A 75-year-old man who had received a diagnosis of idiopathic interstitial pneumonia had been followed in our hospital since 1995, and had been treated with cyclophosphamide since September 1999. He discontinued taking cyclophosphamide without informing us, and two months later he was admitted to our hospital with deterioration of dyspnea on September 13, 2000. Since chest radiography and CT findings demonstrated alveolar infiltrates in the right middle lung field, he was treated with antibiotic agents. Although no deterioration of symptoms occurred, on September 14 he began to suffer rapidly progressive dyspnea accompanied with production of bloody sputum, which eventually developed into full-blown hemoptysis in the evening of September 15. He died of respiratory failure early the next morning. The autopsy findings demonstrated diffuse alveolar hemorrhage, diffuse alveolar damage, interstitial pneumonia, and pulmonary fibrosis.     

Pneumomediastinum and subcutaneous emphysema associated with dermatomyositis

SIR, Dermatomyositis (DM) is a generalized disorder characterizedby myositis and typical cutaneous findings. The lung is frequentlyaffected, usually by interstitial pneumonitis [1]. Spontaneouspneumomediastinum has also been reported as a rare complicationin DM [2–4]. Here we describe a patient with DM who developedinterstitial pneumonitis complicated by pneumomediastinum andsubcutaneous emphysema during steroid therapy. A 28-yr-old man was seen in another hospital in December 1999with complaints of general fatigue, anorexia, fever, symmetricalarthritis of the hands and erythema of the     

Successful treatment of alveolar hypoventilation due to dermatomyositis with anti-tumour necrosis factor-alpha

SIR, Respiratory complications can be seen in the course ofdermatomyositis (DM), but alveolar hypoventilation due to respiratorymuscle weakness has only been infrequently reported [1, 2].Alveolar hypoventilation can be resistant to conventional agents,which may result in either death or dependence on home mechanicalventilation [2, 3]. We present a patient with DM with alveolar hypoventilation whofailed to respond to a combined immunosuppressive treatment.The initiation of anti-tumour necrosis factor-alpha (anti-TNF-)resulted in improvement in her ventilatory insufficiency. A 19-yr-old girl was     

An autopsy case of dermatomyositis associated with interstitial pneumonia probably due to cytomegalovirus infection]

An autopsy case of dermatomyositis (DM) associated with interstitial pneumonia probably due to cytomegalovirus infection is reported. After corticosteroid therapy for 1 month, a 79-year-old man with DM developed acute respiratory failure due to interstitial pneumonia and died in spite of intensive respiratory care. By polymerase chain reaction method (PCR), DNA of cytomegalovirus (CMV) was detected in the bronchoalveolar lavage fluid (BALF). CMV was also detected by the method of conventional virus culture from BALF. These findings suggested that initial infection or reactivation of CMV had occurred in the lungs. The autopsy specimen revealed the findings of interstitial pneumonia compatible with CMV pneumonitis, but without the presence of intranuclear inclusion bodies. Although the present case of interstitial pneumonia should not strictly be diagnosed as definite CMV pneumonitis without the presence of intranuclear inclusion bodies in the lung tissue, initial infection or reactivation of CMV in the lungs may have contributed to the pathogenesis of interstitial pneumonia. In other cases of collagen vascular disease associated with interstitial pneumonia, CMV or other viruses may contribute to the pathogenesis of interstitial pneumonia.     

Death due to diffuse alveolar hemorrhage in a child with pulmonary veno-occlusive disease

Pulmonary veno-occlusive disease (PVOD) occurred in a six-year-old girl. Investigation of a number of proposed causes for this uncommon condition and histologic progression of disease are documented. To our knowledge, this is the first case of PVOD in which death was due to massive alveolar hemorrhage.     

A case of diffuse alveolar hemorrhage associated with interstitial pneumonia and systemic sclerosis]

A 73-year-old female with interstitial pneumonia and systemic sclerosis was admitted with bloody sputum and fever. A chest radiograph showed bilateral infiltrative shadows superimposed on interstitial pneumonia. A chest computed tomograph also showed bilateral airspace consolidations and honeycomb formation. Mechanical ventilation was required because of respiratory failure. At intubation, fiberoptic bronchoscopy revealed bilateral bronchi to be filled with bloody secretions. Her serum myeloperoxidase-anti-neutrophil cytoplasmic antibody titer was slightly elevated. Steroid pulse therapy (1000 mg/day methylprednisolone for 3 days) and cyclophosphamide pulse therapy (500 mg cyclophosphamide) followed by 60 mg/day prednisolone was introduced on the basis of a diagnosis of diffuse alveolar hemorrhage. Her respiratory condition improved and chest infiltrative shadows disappeared and weaning from mechanical ventilation was successful. However, she died of ventilation insufficiency due to progression of her chronic respiratory failure. A necropsy specimen obtained from the left lung showed fibrosis, predominantly in peripheral areas of lobules, and accumulation of hemosiderin-laden macrophages, which findings were consistent with usual interstitial pneumonia and diffuse alveolar hemorrhage. A review of the world literature revealed 4 case reports of diffuse alveolar hemorrhage associated with interstitial pneumonia and systemic sclerosis. We should be aware of this rare but serious complication in the management of interstitial pneumonia associated with systemic sclerosis.     

Severe varicella pneumopathy in health adults. Apropos of a case

The authors report a new case of respiratory distress syndrome which occurred as complication of varicella in an otherwise healthy adult patient. The case is well documented by images and immunological studies which are discussed in the light of recently published data.     

A case of polymyositis with concomitant diffuse alveolar damage following interstitial pneumonia]

A 68-year-old woman had been given a diagnosis of interstitial pneumonia (NSIP pattern) and followed up at our hospital for 3 years. She was admitted to our hospital because of dyspnea, lower limb edema and myalgia. On admission, serum CPK and CRP levels were elevated and an electromyogram suggested inflammatory myopathy. We diagnosed polymyositis (PM) with progressive interstitial pneumonia (IP). Although methylprednisolone pulse therapy and immunosuppressive agents were administered, pulmonary lesions became aggravated. The patient died due to respiratory failure as a result of the progress of IP. The autopsy lung revealed diffuse alveolar damage (DAD) at the both acute and fibrotic phases, suggesting that DAD could coincide with PM. We report here a rare case of polymyositis with diffuse alveolar damage (DAD).     

Severe unilateral diffuse alveolar hemorrhage: an unusual presentation of microscopic polyangiitis

Clinical Rheumatology -     

弥漫性间质性肺疾病的诊断思路

一、概念和分类 弥漫性间质性肺疾病是一组以肺间质炎症和纤维化为主要表现的异源性疾病,因病变主要发生在肺间质,故而得名。目前,许多临床医师对弥漫性间质性肺疾病的概念和分类不清,看到双肺弥漫性病变就笼统归为“肺间质纤维化”,从而导致诊断和治疗的错误,因此有必要对弥漫性间质性肺疾病的概念和分类作一解释。     

Relationship of gold and penicillamine therapy to diffuse interstitial lung disease.

Seven cases of diffuse interstitial lung disease (DILD) are reported with an unequivocal temporal relationship between the development of the lung disease and treatment with gold (6 cases) and penicillamine (1 case). They were characterised clinically by the sudden onset of dyspnoea and crepitations and radiologically by diffuse bilateral pulmonary shadowing. Most showed evidence of hypersensitivity such as eosinophilia, a raised serum IgE level in response to gold, proteinuria, thrombocytopenia, or an immediate postinjection reaction. DILD is a serious complication of treatment with gold and penicillamine that is commoner than generally realised.     

Two cases of autoimmune and secondary pulmonary alveolar proteinosis during immunosuppressive therapy in dermatomyositis with interstitial lung disease

Interstitial lung disease (ILD) with dermatomyositis often requires intensive immunosuppressive therapy. Here, we report two cases of pulmonary alveolar proteinosis (PAP) in dermatomyositis with ILD. One case was secondary PAP, and the other was autoimmune PAP positive for the anti-granulocyte macrophage–colony-stimulating factor antibody. PAP arose during immunosuppressive therapy and symptoms ceased by attenuating immunosuppression. Exacerbation of pulmonary lesions during intensive immunosuppressive therapy may distinguish PAP from worsening ILD and attenuating immunosuppression should be considered.