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KK Krishna*, DK Mitra**, AG Diwan*
*Associate Professor; **Professor and Head, Department of Medicine, Bharati Vidyapeeth Medical College and Bharati Hospital, Dhankawadi, Pune 411 043.

There is a strong association between neoplasms and autoimmune diseases. We present a case of scleroderma with bronchogenic carcinoma.


There is an association between chronic pulmonary fibrosis and development of bronchogenic Ca (either bronchoalveolar cell Ca or adeno Ca) in scleroderma. Carcinoma is thought to arise from intense epithelial proliferation that accompanies the fibrotic process. [1] We report a case who presented with 3 days history of fever and 15 years of arthritis; on investigations was found to have scleroderma and bronchogenic Ca.


A 71 year old female presented with high grade fever and cough since 3 days. She was being treated with NSAIDs for arthritis involving hands since 15 years. On examination, she was pale, febrile, had a pulse of 96/min regular, blood pressure of 150/70 mm Hg, flexion deformities in both hands at metacarpophalangeal, proximal and distal interphalangeal joints (DIP). An ejection systolic murmur grade 3/6 was heard in aortic area conducted to carotids. Bilateral rhonchi, crepitaions in left infraclavicular and right mammary areas were present. Her haemoglobin was 10 g%, ESR 45, WBC count 11,600/cmm (P 85%, L 10%, M 1%, E 4%), MP negative, platelet count 92,000/cmm, hypochromic microcytic anaemia, stool positive for occult blood; RA factor, LE cell, ANA negative, ECG - sinus tachycardia, X-Ray hands-generalised osteoporosis with destruction and deformities at DIP. Plain radiograph (Fig. 1) and CT scan of chest (Fig. 2) was suggestive of consolidation in left upper lobe and apical segment right lower lobe. Bronchoscopy revealed inflammation and ulceration in apicoposterior segment of left upper lobe and partial blockade of right lower bronchus with irregular ulcerated and oedematous mucosa. Bronchial biopsy was diagnostic of squamous cell carcinoma. Skin biopsy from fingers was suggestive of progressive systemic sclerosis.

Fig 1 : Plain radiograph
Fig 2:  CT scan of chest
Fig 1
Fig 2
Plain radiograph and CT scan of chest respectively showing consolidation in apical segment of the right lower lobe and anterior segment of the left upper lobe. Whiskered margins of the lesions with thin fibrotic strands radiating around the lesions are seen.

The patient was treated with antibiotics (Inj. Cefotaxime 1 g IV 8 hourly) for 7 days. She became afebrile on day 3. She was referred for chemotherapy for the malignancy.


Scleroderma is a rare disease with limited data on its occurrence. The reported prevalence rate is 30-120/million/year population, annual incidence of new cases is lower (2-20/million/year). Rates are substantially higher in females with a peak onset in the 5th and 6th decade. There is an increase in cancer mortality. [2] Overall 5 and 10 year survival rate is reported 86% and 69% respectively. There is a 4.6 fold increased risk of death as compared to general population. Most patients die of cardiopulmonary disease. Prognosis is worse in diffuse involvement and male subgroups. In a study of 249 patients followed up for 13 years there were 49 deaths of which 24 were attributed to pulmonary complications - pulmonary fibrosis, pulmonary hypertension, pneumonia or pulmonary malignancy. Of the 7 fatal lung cancers, 5 were adeno Ca, possibly caused by chronic inflammatory disease of the lung. [3] Our patient had a squamous cell Ca.

In a study of 21 cases of scleroderma with associated Ca, neoplastic localisation were the following - pulmonary Ca-5, breast Ca-2, 1 each of oesophageal, stomach, colon, uterus, ovarian, prostatic, renal Ca and 6 cases of malignant haemopathies. Recent epidemiological studies allow to conclude to a higher frequency of lung and breast Ca. Patients with systemic scleroderma should be examined attentively and carefully for these risks. [4]

There is a strong association between neoplasms and autoimmune diseases. Numerous autoimmune phenomena have been reported in malignancies and conversely : malignant tumours are diagnosed with increasing frequency in autoimmune conditions. [5] Anti DNA topoisomerase I antibody levels determined by double immuno-diffusion and ELISA are markedly elevated in patients of scleroderma at the time of diagnosis of lung cancer. [6] In another study of 123 patients with systemic sclerosis 14 cases of cancer were found; 3 cases of lung cancer in association with CREST syndrome and anti centomere antibodies were reported. [7]

There have been reports of lung cancer occurring both in close spatial relation to localised areas of pulmonary scarring and in patients with more diffuse lung fibrosis.

Systematic microscopic examination of lung scar tissue has revealed areas of epithelial hyperplasia in the vicinity of the scarring and this in turn may be associated with unexpected carcinomatous change. The pathogenesis of the relatively small proportions of the scar cancers is not established and in many cases the subjects tobacco smoking history may be highly relevant. [8]

To summarise, the salient features of this case were (1) short duration of presenting symptoms of cough and fever (2) arthritis of small joints of hand since 15 years (3) severe anaemia (4) radiograph and CT scan of chest suggestive of bilateral consolidation (5) bronchial biopsy reporting squamous cell carcinoma (6) skin biopsy suggestive of scleroderma.


  1. King TE, Cherniack RM, Schwarz MI. The lungs and connective tissue diseases In: Textbook of Respiratory Medicine 2nd edn. Murray JF, Nadel JA eds. WB Saunders London. 1994; 1850-72.
  2. Silman AJ. Scleroderma - dermographics and survival. Journal of Rheumatology - Supplement 1997; 48 : 58-61.
  3. Hesselstrand R, Scheja A, Akesson A. Mortality and causes of death in a Swedish series of systemic sclerosis patients. Annals of the Rheumatic Diseases 1998; 57 (11) : 682-86.
  4. Biefeld P, Meyer P, Caillot D, Dalac S, Camus P, Tavernier C, Couillaut G, Chalopin JM, Besancenot JF. Systemic scleroderma and cancers : 21 cases and review of the literature. Revue de Medicine Interne 1996; 17 (10) : 810-13.
  5. Tomer Y, Sherer Y, Shoenfeld Y. Autoantibodies, autoimmunity and cancer (review). Oncology reports 1998; 5 (3) : 753-61.
  6. Kuwana M, Fujii T, Mimori T, Kab uraki J. Enhancement of anti DNA topoisomerase I autoantibody response after lung cancer in patients with systemic sclerosis. A report of two cases. Arthritis and Rheumatism 1996; 39 (4) : 686-91.
  7. Kyndt X, Hebbar M, Queyrel V, Hachulla E, Hatron PY, Devulder B. Systemic scleroderma and cancer. Search for predictive factors of cancer in 123 patients with scleroderma. Revue de Medicine Interne 1997; 18 (7) : 528-32.
  8. Seaton A, Seaton D, Leitch AG. Cancer of the lung. In : Seaton A, Seaton D, Leitch AG eds. Crofton and Dougla’s Respiratory diseases 4th ed. Blackwell Science Ltd, Oxford. 1997; 912-74.

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