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Scleroderma is one of the causes of lower lobe pulmonary fibrosis

Posted Sep 11 2009 4:56pm

This is used to describe both systemic sclerosis and morphoea. The former is a multi-system disorder whilst the latter is a purely cutaneous disease. Hardening of the skin is a common feature but unlike the variants of lupus erythematosus, the two dermopathologies involved in scleroderma are different. Both primary disease processes are essentially untreatable.

A case of a 52 year old female with scleroderma, pulmonary fibrosis and recurrent acute respiratory distress

INTRODUCTION: Categorizing interstitial pneumonia can be a challenge for physicians. We present a 52 year old female with scleroderma and associated lung disease who manifested recurrent acute illness resembling acute interstitial pneumonitis.

CASE PRESENTATION: A 52 year old female with a two year history of scleroderma including esophageal dysmotility, morphea, Raynaud’s, telangiectasias, sclerodactyly, and pulmonary fibrosis without pulmonary hypertension and with normal LV function presented with repeated hospitalizations over a period of a year with acute respiratory distress. At the initial hospitalization, the patient presented with increasing shortness of breath and dyspnea on exertion. Vital signs, including oxygen saturation, were normal. A chest CT revealed ongoing lower lobe pulmonary fibrosis. The patient, who was on prednisone 60mg, was treated with intravenous steroids and quickly improved. One month later, while on low dose steroids, the patient developed acute respiratory distress. Chest radiograph revealed "diffuse bilateral opacities". Her respiratory rate was 30, heart rate was 147, BP was 140/70. The patient was afebrile with a normal WBC count. An ABG revealed 7.17/36/43/13/64%. The patient was intubated and transferred to the ICU. IV steroids and broad-spectrum antibiotics were started. On the same day, a bronchoscopy with BAL was negative for legionella, AFB, fungal, PCP, CMV, adenovirus, VZV, and HSV. Blood cultures were negative. The patient quickly improved and was extubated. Again a month later, the patient developed hypoxic respiratory failure requiring intubation. A chest radiograph revealed "extensive air space disease", a chest CT with contrast showed, "extensive interstitial lung disease at both bases" but also revealed new "ground-glass opacities in the upper lobes". Again a BAL found no infectious etiology. IV steroids and broad-spectrum antibiotics were restarted. An open-lung biopsy was obtained. Right middle lobe tissue revealed "end-stage lung with extensive fibrosis and honeycombing ...also mild focal chronic interstitial inflammation". Special stains were negative for organisms. The patient improved, and was eventually discharged from the hospital. Finally seven months later, the patient again presented with hypoxic respiratory failure requiring intubation. Chest radiograph revealed "diffuse airspace consolidation". The patient was afebrile with a normal WBC. IV steroids and broad-spectrum antibiotics were given. A BAL was negative for infection. The patient’s oxygenation and chest radiograph rapidly improved. The patient was soon extubated. 6 days later the patient’s shortness of breath increased. Unlike previously, the patient began to have fevers and increased WBC. A repeat BAL revealed multiple organisms. A chest CT again revealed the previously new upper lobe interstitial changes, but also showed air bronchograms in the lower lobes. The patient continued to deteriorate, became progressively hypoxic, and later expired. The autopsy revealed "extensive interstitial fibrosis, diffuse alveolar damage, multifocal areas of organizing pneumonia" as the cause of death.

DISCUSSIONS: Over 70% of patients with scleroderma develop pulmonary disease. There are two usual manifestations: pulmonary fibrosis and pulmonary hypertension. The pulmonary fibrosis is slowly progressive, lower lung zone predominant, and similar in character to UIP. This patient is unique in that she had two separate but coexisting lung processes: scleroderma related disease, and a fulminant diffuse process. This process clinically presented as idiopathic recurrent ARDS without systemic involvement, and pathologically showed diffuse alveolar damage. Although, rarely RA and polymyositis have had a Hamman-Rich type syndrome associated with them, scleroderma has not. We, therefore, propose this as a rare case of scleroderma associated with an AIP-type syndrome.

CONCLUSION: With reference to the ATS consensus statement, this case exemplifies an "area of uncertainty" surrounding the interstitial pneumonias. In this statement, a connective tissue disease such as scleroderma excludes a diagnosis of AIP. Uncertainty presents itself in such issues of overlap challenging proper diagnosis, perhaps affecting treatment, and leaving questions as yet unanswered.

The other causes of lower lobe pulmonary fibrosis such as:

rheumatoid arthritis
Rheumatoid arthritis is a chronic systemic disease that primarily affects the joints. Presentation is typically as an insidious polyarthritis characterised by inflammatory changes in the synovial membranes and articular structures leading to deformity and ankylosis. Systemic features usually develop as the disease progresses.

Asbestosis is a pneumoconiosis caused by inhalation of asbestos fibres, classically causing a chronic, diffuse, interstitial fibrosis of the lung. Crocidolite - blue asbestos - is the most important type of asbestos in the aetiology of asbestosis and particularly, pleural and peritoneal mesothelioma.

Asbestos exposure increases the risk of bronchogenic carcinoma 5-fold. In exposed cigarette smokers, the risk is increased 55-fold. An asbestos-related increase is also observed in the incidence of gastric, colonic and renal adenocarcinoma, and of gastrointestinal lymphomas.

Under the UK Social Security Act of 1975 various conditions caused by asbestos exposure are eligible for industrial compensation, including bilateral diffuse pleural thickening, asbestosis, mesothelioma and asbestos related carcinoma of the bronchus.

Pneumoconiosis is a term used to signify the accumulation of dust in the lungs and the reaction of the tissue to its presence. It is now generally restricted to the effects of coal dust and is also known as coal worker's pneumoconiosis.

The particles are approximately 2-5 micrometres in diameter and are retained in the small airways and alveoli of the lung.

The development of pneumoconiosis is directly related to total exposure to coal dust. The highest incidence of this disease is in miners who work at the coal face, especially those who work in poorly ventilated conditions.

Historically, pneumoconiosis occurred in about 12% of all coal miners and in up to 50% of those who mined anthracite for more than 20 years. There has been a dramatic reduction in incidence to about 2 per 1000, due to improved health and safety standards in the coal industry.

Paraquat is a poisonous dipyridilium compound whose dichloride and dimethylsulphate salts are used as a contact herbicide - the most common of which are "Weedol", "Pathclear", and "Gramoxone." Most cases of poisoning are deliberate, and the sooner treatment is commenced, the better.

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