© JAPI • mArch 2011 • VOL. 59 175

aneurysm by facilitating complete thrombosis of false lumen. The diameter of stent graft should be 2-6 mm or 20% bigger than the diameter of the proximal normal aorta and the length of the stent graft varies from 10-12 cm. It is important to mention here is the fact that sealing of the entry point of dissection is important while deploying a stent graft and the length of the length graft need not be as long as the dissected segment or aneurysm because thrombus will soon form in the false lumen after the stent graft intervention Excessive stent graft may exclude many intercostal arteries from the true lumen and increases the possibility of paraplegia.

In our case the patient tolerated the procedure very well with no local or systemic complication.

In conclusion, endovascular stent graft offers lesser invasive technique for treating aortic dissections with good results(almost 93% success rate ) and lesser complications viz. spinal cord ischaemia, cardiac and pulmonary complications and should be the procedure of choice if anatomy is suitable and expertise is available.

References1. The Gale Encyclopedia of Medicine. 3rd ed. Stamford, conn: Gale 2008 .2. Slater EE, DeSanctis rW. The clinical recognition of dissecting aortic

aneurysm. Am J Med 1976;60:625-633.3. Parodi Jc, Palmaz Jc, Barone hD. Transfemoral intraluminal

graft implantation for abdominal aortic aneurysms. Ann Vasc Surg 1991;5:491-499.

4. Kato N, Dake mD, miller Dc, et al. Traumatic thoracic aortic aneurysm: treatment with endovascular stent-grafts. Radiology 1997;205:657-662 .

5. André Nevelsteen. Endovascular treatment for thoracic aortic dissection: the better solution? European Heart Journal 2006;27:384-385.

6. Duhaylongsod FG, Glower DD, Wolfe WG. Acute traumatic aortic aneurysm: the Duke experience from 1970 to 1990. J Vasc Surg 1992;15:331-342.

into IIIa and IIIb. 3a. Type IIIa refers to dissections that originate distal to

the left subclavian artery but extend both proximally and distally, most above the diaphragm.

3b. Type IIIb refers to dissections that originate distal to the left subclavian artery, extend only distally and may extend below the diaphragm.

Ever since Parodi et al3 described their first clinical experience with use of a stent-graft to treat an abdominal aortic aneurysm, endoluminal stent-graft placement has been emerging as a less invasive alternative to conventional surgery in patients with aortic aneurysms or aortic dissections and with serious coexisting illnesses.4 Currently stent grafts are now used with confidence in the management of wide variety of thoracic aortic pathologies including acute and chronic dissection, intramural haematoma, penetrating ulcers, traumatic injuries.

The technical success of endovascular treatment is over 95% with a 30-day mortality rate of 5.3%. major complications might be seen in one out of ten patients with endoleak being the most common complication. Endoleak can cause progressive enlargement of aortic dissection and even rupture. It needs additional stenting with preferably a bigger sized stent .If it fails again then surgery is the answer. The incidence of neurological complications is quite low: peri-operative stroke occurs is less than 3% of the cases and the incidence of paraplegia is less than 1%.5

Endoluminal repair of type B dissection can obviate thoracotomy, which may cause respiratory problems, especially in patients with preexisting pulmonary insufficiency. Another advantage of the stent-graft procedure over conventional surgery is the absence of aortic clamping that may contribute to major complications, such as failure of the left side of the heart or paraplegia. It remains debatable whether cross-clamping time alone can be used to predict the relative risk of paraplegia. There is no doubt, however, that cross-clamping is associated with the risk of paraplegia.6 Aortic stent graft prevents the devlopment of

*consultant Pulmonologist, **Senior consultant Pulmonologist, ***Senior consultant Thoracic Surgeon, †Senior registrar, Dept of Internal medicine, ‡consultant Physician, Dept of Internal medicine, #Professor and head, Dept of Internal medicine, Kerala Institute of medical Sciences (KImS), Trivandrumreceived: 23.07.2009; Accepted: 29.09.2009

Undifferentiated Connective Tissue Disease with Pulmonary InvolvementP Arjun*, KA Ameer**, S Sasikumar***, A Rajalakshmi†, TA Hari‡, Mathew Thomas#

AbstractPulmonary involvement in collagen vascular diseases is extremely common. It is usually seen in the well described dyscollagenoses and in mixed connective tissue diseases (mcTD). however, there is a lesser known entity called Undifferentiated connective Tissue Disease (UcTD) which can also involve the lung. We herein present a case of a young man who was detected to have lung involvement secondary to UcTD

Introduction

Diagnosis of connective tissue diseases is often delayed because the initial symptoms are few and non-specific.

Often they have multi system manifestations and lung is one of

the most commonly affected organ. Lung involvement can range from consolidation, interstitial pneumonitis, to pleural effusion. rarer manifestations include pneumothorax, and bronchiolitis obliterans. A newer entity called Undifferentiated Connective Tissue Disease (UcTD) has been recently described, in which the patient has signs and symptoms of a collagen vascular disease but does not fulfill the criteria of any of the described entities.1 It is usually a diagnosis of exclusion. We report the case of a young man who presented to us with fever, chest pain and myalgia, who on detailed work up was detected to have UcTD with lung involvement in the form of Non Specific Interstitial Pneumonia

176 © JAPI • mArch 2011 • VOL. 59

(NSIP) – an interstitial lung disease. The lung involvement in our case has been with an atypical presentation of NSIP in the form of pulmonary nodules. To the best of our knowledge there has not been any reported case of UcTD with lung involvement from India till date.

Case ReportA 35 year old man employed as a welder in an oil field outside

India, presented to our hospital with fever and right sided chest pain, suggestive of pleuritic pain of 2 month’s duration. he did not have cough, wheeze or breathlessness. he gave history of having recurrent episodes of constitutional symptoms, myalgia and chest pain for the past 5 years. he was diagnosed as having fibromyalgia from another hospital and was started on oral prednisolone 30 mg per day. he did not report for follow up and was self medicating with 10 mg prednisolone off and on, with which he had some symptomatic relief. He had first presented to us one year prior to this admission with right sided chest pain. A chest X ray taken at that time had revealed the presence of right sided pleural effusion. He was worked up for dyscollagenosis and ANA, anti ds DNA and rheumatoid factor were negative. He had a positive tuberculin test, pleural fluid ADA was raised and skin nodules which resembled erythema nodosum and hence was started on empirical ATT, which he continued for 6 months. he became totally asymptomatic and went back to resume his job.

At admission this time, he was ill looking, had a temperature of 102°F, heart rate of 100/min and BP of 110/70 mm hg. his respiratory system was normal on clinical examination. Other systems were also normal on examination. his laboratory investigations were as follows: hemoglobin 12.2g%, Tc 20400, P 88%, L 10%, E 2%, ESr 108 mm/hr and peripheral smear showed neutophilic leukocytosis. X ray chest PA view showed multiple nodular opacities, predominantly in the upper and mid zones bilaterally. Sputum Gram stain showed Gram positive cocci and Gram negative bacilli and cultures grew Klebsiella, following which appropriate antibiotics were started. AFB smears were

Fig 1: HRCT Thorax showing multiple, discrete, well-defined, irregular peribronchial nodules

negative for 3 days. Blood cultures did not show any growth. his LFT and rFT were normal. USS abdomen was unremarkable except for a left renal cyst. his lung function was suggestive of a restrictive pattern (FVC 45%, FEV1 45%, FEV1 100%). EcG was normal. his crP was elevated (230 mg/L), rheumatoid factor was positive whereas ANA, ds DNA, c ANcA, p ANcA and ELISA for hIV were all negative. A high resolution cT scan of the thorax was done, which showed multiple, discrete, well-defined, irregular peribronchial nodules predominantly involving both upper lobes (Fig 1). The radiological differential diagnosis considered were invasive aspergillosis, disseminated TB, Lymphoma and PcP. he was then worked up to rule out the above mentioned conditions. Sputum fungal stain was negative and fungal culture had no growth. Giemsa staining for PcP was negative. Sputum cytology did not show any malignant cells and TB PCR in sputum was negative. A flexible bronchoscopy was done which was normal. Bronchial Lavage and Trans bronchial biopsy were negative for AFB, fungal elements and malignant cells. he still continued to have spiking fever and his chest pain was worsening even after parenteral antibiotics.

Since no diagnosis could be reached till now, he was offered an option of open lung biopsy, for which he agreed. He underwent a left mini thoracotomy and multiple biopsies were taken from left upper lobe and linguala. histopathologically it was a diffuse, chronic interstitial pneumonia with foamy macrophages, areas of fat droplets and organising pneumonia like pattern in some areas (Fig 2).

There was no evidence of fungal infection, TB, lymphoma or PCP. The final histopathological impression was “Lipoid pneumonia with areas of organising pneumonia and chronic interstitial pneumonia”. A second opinion was obtained from two other tertiary centres specializing in pulmonary pathology. They also opined that the histopathological features were suggestive of a diffuse lung disease with lipoid pneumonia. Welder’s lung, an occupational lung disease was ruled out by polarising microscopy. Putting together all the clinico-pathologico-radiological findings, it was certain that our patient had a “sub-acute interstitial pneumonia with features consistent with NSIP”. The final diagnosis was Non Specific Interstitial Pneumonia secondary to Undifferentiated Connective Tissue Disease.

Fig 2: Histopathology (H&E, X 400) showing diffuse lymphoplasmacytic infiltration, foamy macrophages and interstitial

fibrosis.

© JAPI • mArch 2011 • VOL. 59 177

DiscussionOur patient was initially suspected to have an infectious

cause for his fever and constitutional symptoms in view of his X- Ray appearances and raised blood counts. The CT findings then led to an evaluation for TB, fungal infection, PcP and lymphoma. These conditions were considered because he was immunocompromised in view of his self medication with oral steroids. The findings on open lung biopsy were helpful in ruling out all these conditions. The histopathological diagnosis of lipoid pneumonia raised certain other possibilities. Lipoid pneumonia is a noninfectious, inflammatory lung disorder caused by the aspiration of mineral oil or other oily substances. It is classified as exogenous and endogenous. Exogenous is usually due to aspiration of oil, inhalation of oil droplets and use of oil-based laxatives or oil based nasal drops. Occupational exposure occurs in furniture factories and steel mills and lipoid pneumonia is seen as an occupational hazard in fire eaters and is called “fire eater’s lung”.2 Endogenous lipoid pneumonia on the other hand is seen in bronchogenic carcinoma, Undifferentiated Connective Tissue Disease, Diffuse panbronchiolitis, Bronchiectasis, Amiodarone lung toxicity and Pulmonary hypertension.3 It mimics bronchogenic carcinoma and fungal infections. Putting together the clinical, radiological and histopathological findings and considering the unique aspects of our case we made a diagnosis of Endogenous lipoid pneumonia secondary to Undifferentiated connective Tissue Disease.

The term Undifferentiated connective Tissue Disease (UcTD) is a relatively recent entity suggested by Leroy et al in 1980 and is different from Mixed Connective Tissue Disease (mcTD).4 UcTD is a condition in which the patient has signs and symptoms suggestive of a connective Tissue Disease for at least three years, but not fulfilling the criteria for any of the defined dyscollagenoses. The diagnostic criteria for UcTD is as follows: at least one clinical manifestation of connective tissue disease, serologic evidence of systemic inflammation in the absence of clinical infection, and absence of sufficient American College of rheumatology criteria for another connective tissue disease(5). It

is thought that UcTDs represent around 60% of diseases with an undifferentiated onset, that they are systemic autoimmune diseases characterized by simplified clinical and serological profiles, and that they have a good prognosis.1 Our patient had just two of the criteria for rheumatoid arthritis - rheumatoid nodules and elevated serum rheumatoid factor, did not meet the criteria of any other dyscollagenosis and thus fulfilled the criteria for the diagnosis of UcTD. A small percentage of patients presenting with an undifferentiated profile will develop during the first year follow up of a full blown CTD, however an average of 75% will maintain an undifferentiated clinical course. These patients may be defined as having a stable undifferentiated connective tissue disease (UCTD.5 The most characteristic symptoms of UcTD are represented by arthritis and arthralgias, raynaud’s phenomenon, leukopenia, while neurological and kidney involvement are virtually absent. Skin involvement is common and the manifestations include Telangiectasia, purpura, erythema nodosum, subcutaneous nodules, petechiae, sclerodactyly, malar rash, discoid rash, calcinosis and acroscleroderma. The pulmonary manifestations of UcTD include dyspnea, orthopnea, cough, wheezing, pleuritic chest pain, rales, wheezing, pleural effusion and pleural rub. Our patient had erythema nodosum, subcutaneous nodules and pleuritic chest pain. After obtaining second opinion on the histopathology slides, it was evident that the lung involvement was of a NSIP (non specific interstitial pneumonia) pattern.

NSIP has been recognized as a major histopathologic

pattern of lung involvement in scleroderma, polymyositis-dermatomyositis, or Sjögren’s syndrome. In addition, in some patients, lung involvement precedes other systemic manifestations, making the distinction between idiopathic NSIP and lung involvement of connective tissue disease impossible at the time of diagnosis. hrcT features of NSIP are varied and include ground glass opacities (100%), Irregular linear opacities (46%), Traction bronchiectasis (36%), consolidation (16%), Nodular opacities (14%) and Emphysema (12%). Our patient had the nodular form of opacities in cT. There is now an emerging school of thought that NSIP is an autoimmune disease and the pulmonary manifestation of undifferentiated connective tissue disease.6

Treatment and Follow UpOur final diagnosis was NSIP secondary to Undifferentiated

connective Tissue Disease. The patient was started on Oral prednisolone 30 mg /day and low dose methotrexate. he made a dramatic recovery, became totally asymptomatic and was able to go back and resume his job. On follow up 1 year later his cT scans showed near complete clearance of the lesions (Fig 3). his lung function studies also demonstrated a remarkable improvement with a FEV1, FVc and FEV1% one year later. he is at present on a low dose of Prednisolone (5mg on alternate days) and methotrexate.

AcknowledgementWe sincerely thank Prof V G chellam, chief Pathologist

KImS and Prof. S Shankar, head, Dept of Pathology, medical college, Trivandrum for their valuable help with histopathology interpretation.

References1. Mosca M, Tani C and Bombardieri.S. Undifferentiated connective

tissue diseases (UcTD): a new frontier for rheumatology. Best Practice & Research Clinical Rheumatology 2007;21:1011-1023.

Fig. 3: HRCT Thorax one year later showing near complete resolution of lesions

178 © JAPI • mArch 2011 • VOL. 59

2. Kitchen JM, O‘Brien DE, McLaughlin AM. Perils of fire eating. An acute form of lipoid pneumonia or fire eater‘s lung. Thorax 2008;63:401-439.

3. Barta Z, Szabo GG, Bruckner G, Szegedi G. Endogenous lipoid pneumonia associated with undifferentiated connective tissue disease (UcTD) med Sci monit. 2001;7:134-6.

4. LeRoy EC, Maricq HR, Kahaleh MB. Undifferentiated connective tissue syndromes. Arthritis Rheum 1980;23:341-3.

5. Mosca M, Neri R, Bombardieri S. Undifferentiated connective tissue diseases (UcTD): a review of the literature and a proposal for preliminary classification criteria. Clin Exp Rheumatol 1999;17:615-20.

6. Kinder BW, collard hr, Koth L, Daikh DI, Wolters PJ, Elicker B, Jones KD, King TE Jr. Idiopathic nonspecific interstitial pneumonia: lung manifestation of undifferentiated connective tissue disease? Am J Respir Crit Care Med 2007;176:691-7.

*Associate Professor of medicine, **Senior Professor of medicine and Pro-Vice chancellor, ***resident in medicine, ****Associate Professor of Bio-chemistry, Dept. of medicine and Biochemistry, Pt. BD Sharma PGImS, rohtak, haryanareceived: 08.10.2009; revised: 09.12.2009; Accepted: 11.12.2009

Reversible Atrioventricular Blocks in Thyroid StormSudhir Kumar Atri*, SN Chugh**, Sandeep Goyal***, Kiran Chugh****

AbstractAtrioventricular blocks or sinoatrial blocks are rarely described in patients with thyrotoxicosis or thyroid storm. The mechanism of these blocks remains obscure. Thyroid storm, being an emergency situation requires early diagnosis and management because if left untreated, it may prove fatal. Usually patients with AV blocks require pacing (temporary or permanent). here we describe a case who developed AV blocks, did not undergo pacing, but recovered only on antithyroid treatment.

Introduction

Thyroid hormones are known to have direct effect on the intrinsic SA node electrophysiological function. Thyrotoxicosis is

usually associated with accelerated conduction through AV node. conduction disturbances are rare; occur in patients with severe or prolonged thyrotoxicosis. These disturbances have been reported as case reports in the literature1-2, the mechanism of which still remain unexplained. concomitant secondary factors such as infection3 (myocarditis), drugs (beta blockers) and electrolyte disturbances have been blamed in most of the cases; isolated thyrotoxicosis has been postulated as a cause in few cases. here, we describe a patient who presented with thyroid storm and varying degree of heart blocks without atrial fibrillation which reversed with supportive and antithyroid treatment. The presentation of AV blocks in a freshly diagnosed untreated patient with thyroid storm is a rare phenomenon.

Case Report60 year old female developed profuse diarrhea and vomiting

for 3 to 4 days, became dehydrated and went into shock. She was admitted outside this hospital where she was given 8 to 10 liters of I.V. fluids, Following this, she complained of shortness of breath and swelling feet and was hospitalized at PGImS rohtak. At admission there was history of dyspnoea, othopnoea, PND and syncopal attack with profuse perspiration. There was no history of abdominal pain, distension and fever. There was also no history to suggest any cardiac disease in past.

Examination revealed a thin lean female with signs of fluid overload, slow bounding pulses, wide pulse pressure (BP -180/60 mm hg) and pedal edema. Neck examination revealed raised JVP and multinodular goiter with audible bruit over it. The skin was moist and warm. There were fine tremors over outstretched hands which made us suspicious that she might be a case of toxic goiter. Auscultation of the heart demonstrated loud S1 with short systolic murmur over the apex and pulmonary area.

Investigations revealed hb 8.5g%, TLc 5000 cu/mm, DI.c-60, 35, 2, 2, 1.Liver function tests were normal. Electrolytes (Na+

145 meq/L, K+ 4.5 meq/L) were normal. chest X ray showed prominent cardiac shadow with prominent bronchovascular markings. Serum T3, T4 were high i.e. 364 ng/dl and 16.3 mcg/dl (normal values being 60-120 ng/dl and 0.25-5 mcg/dl respectively) and TSh was low i.e.0.05 IU/ml (normal value-0.95-2.5 IU/ml).There were no antithyroid antibodies. USG showed multinodular Goiter. EcG at admission showed 2:1 and complete AV blocks (Figs. 1, 2). Echocardiogram revealed EF > 65% without any segmental abnormality. Patient was put on diuretics, steroids and antithyroid treatment with monitoring of vitals. Temporary pacing was advised. Patient left the hospital against medical advice but continued the antithyroid treatment herself. To our great surprise, patient came for follow up at 4 weeks. EcG was repeated which was reported to be normal (Fig. 3). Thyroid function repeated after 6 weeks were within normal limits indicating euthyroid status.

DiscussionAlthough cardiovascular manifestations are presenting

features in hyperthyroidism, but atrioventricular conduction disturbances are rarely encountered.1-2 Various grades of AV blocks especially complete A.V. block in hyperthyroidism often occur in the presence of other associated conditions i.e. infection, drugs, hypokalemia and congestive heart failure. In some cases, there is no identifiable cause and hyperthyroxinemia,

Fig. 1 : ECG showing 2:1 AV block

Fig. 2 : ECG showing atrial tachycardia and complete heart block with atrial rate of 124 and ventricular rate of 46 per minute