P65 Spontaneous carotico-cavernous fistula in ehler danlos syndrome

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Indian Journal of Rheumatology 2010 November; Vol. 5, No. 3 (Suppl)

space without affecting femur head suggestive of fibrous dysplasia. Radionuclide bone scan (Figure 3) confirmed localized increased uptake in left iliac bone. Bone biopsy (Figure 4) was consistent with Paget’s disease. Discussion: It is not fibrous dysplasia because of very high alkaline phosphatase level, increased uptake in bone scan and biopsy features consistent with Paget’s disease. Patient was treated with intravenous Ibandronate, as most of the conventional bisphosphonates are contraindicated in renal failure. Conclusion: Mono-ostotic Paget’s disease is a very rare condition described in India. This condition needs to be differentiated from fibrous dysplasia with the help of serum alkaline phosphatase, bone scan and bone biopsy. Presence of renal failure limits the treatment options available for this potentially dangerous disease.

P63 Sneddon’s syndrome AP Naveen Kumar, Mala Saranathan, Manisha Thakre, Liza Rajasekhar Visakha Steel General Hospital, Visakhapatnam and NIMS, Hyderabad Introduction: Generalised Livedo racemosa or Livedo Reticularis with cerebrovascular lesions, that cause focal neurological symptoms and signs is SNEDDON’S SYNDROME. While Livedo Reticularis is the initial manifestation, other clinical findings like headache seizures, visual defects, focal paresis and hypertension may follow. Case Presentation: A 33 year old female patient presented with sudden onset of weakness of both upper limbs and lower limbs, involuntary movements of the limbs, weakness of left half of the face. No h/o fever, cough, cold, LOC, syncope, incontinence, head injury, no drug intake. On examination she was conscious and coherent, no edema, pallor, cyanosis and clubbing. She was Tachycardic, BP-200/130 mm of Hg in both upper limbs, all PP-felt, no radiofemoral delay and no renal bruit. CVS-S1, S2-normal, no murmurs. Lungs-clear. CNS Higher functions normal, left facial UMN palsy, reduced power in the distal muscles of right upper limb, Power of 4b/5 in both lower limbs and fine involuntary movements of both upper limbs Plantar-Extensor (Rt). No sensory deficit or signs of meningeal irritation. Co-ordination was difficult. Past history was significant in view of erythema of the face and hands on and off since 2 years and photosensitivity, no h/o oral ulcers, joint pains, or Raynaud’s phenomenon, not a hypertensive or diabetic. she was married, 2 FTND, H/O PIH during both the pregnancies, no h/o Abortions. She had Malar flush and Telangiectasia over malar region. Livedo Reticularis over both fore arms, no purpura, no Telangiectasia of the nailfolds, cuticles normal. Investigations were insignificant as far as biochemical tests were concerned. MRI of the Brain showed multiple lacunar infarcts. Patient was treated with Anti hypertensives, Anticoagulants, and systemic steroids. Steroids were rapidly tapered off and she is on anti hypertensives and acitrom and doing well with no residual neurological defects. ANA, DSDNA, APL Antibodies were negative. Diagnosis of Sneddons was considered in view of livedo reticularis and CNS manifestations. Over a period of 10 days hypertension got controlled, facial palsy, muscle weakness improved, and choreoathetoid movements disappeared. Patient is on regular follow up in our hospital and Department of Rhematology, NIMS and doing well.

Poster presentations Spontaneous Carotico-Cavernous Fistula (CCF) is a very important complication of connective tissue diseases especially Ehler Danlos syndrome (EDS) TypeIV. It requires emergency intervention in the form of coiling via the nonconventional transvenous route to avoid risk of arterial dissection and rupture. We present two cases who presented to with unilateral painful red eyes with proptosis and external opthalmoplegia with severe visual loss. There was no family history of similar illness. MRI Scan of the Orbit revealed enlargement of the cavernous sinus with prominence of the superior ophthalmic vein which was suggestive of Carotico-Cavernous Fistula. The diagnosis was confirmed with a Carotid arteriogram. Physical examination showed hyper mobility of the small joints greater than large joints, hyperelastic skin, atrophic scarring in both the cases. A diagnosis of Ehler Danlos syndrome (EDS) Vascular type IV was considered. A genetic was planned but could not be performed because of financial constraints. Both of the patients underwent closure of the fistula with transvenous deposition of micro coils in cavernous sinus. Spontaneous CCF can be a presenting manifestation of EDS Type IV. A diagnosis of this condition is crucial because of the difference in management. Family history, Careful physical examination of skin and joints and genetic testing can give a clue regarding the underlying etiology.

P66 Rare case of tubercular monoarthritis with antiCCP positivity Vikram Haridas, Prakash Sanknur, CS Choukimath, Arthritis Superspeciality Center, Hubli Background: Tuberculosis is a common cause for monoarthritis The anti-cyclic citrullinated peptide (anti-CCP) enzyme-linked immunosorbent assay (ELISA) has high sensitivity and specificity for rheumatoid arthritis (RA). Presence of antiCCP in high titers is associated with erosive and severe rheumatoid disease. Its presence in early arthritis helps to identify very early rheumatoid arthritis. Recent studies report antiCCP positivity in active pulmonary tuberculosis. We present a rare case of tubercular monoarthritis of ankle with high titer antiCCP. Case: 39 year old male had a h/o pain and swelling in the right ankle joint since one yr. He didn’t have pain in any other joints. No h/o fever/low back pain/skin rash/oral ulcer/heel pains/loose stools/loss of appetite/family h/o tuberculosis. His blood investigation showed normal hemogram except for raised ESR-100 mm/1 hr and CRP-58 mg/dl. RF-178 mg/dl Anti CCP-332ru/ml, negative montuax test and normal chest X ray. His recent x ray of the ankle joint showed lot of destruction with loss of joint space. His joint CT showed erosion in tibia, fibula with loss of joint space suggestive of tuberculosis. Biopsy of the joint showed granulomatous lesion in the sample available s/o tubercular arthritis. At present patient is on Anti tubercular treatment. Anti CCP which is highly specific for RA can mislead as in this case. Since Tuberculosis is very common in the subcontinent role of antiCCP in the diagnosis of RA should be reconsidered. Conclusion: Role of anti CCP in diagnosis of rheumatoid arthritis should be reconsidered, as its also seen in tuberculosis.

Conclusion: This case is being presented because of its clinical presentation and its rarity and good recovery.

P67 Initial joint involvement in Indian patients with rheumatoid arthritis (RA) and its relationship with response to drug treatment

P64 Early onset interstitial lung disease and JO 1 positivity

Sharath Kuma, Roopa Rawat, S Sadhana Baghel, Dr AN Malaviya

Praveen Kumar Yadav, C Jayakumar, Jacob Anthony

Background: Large joint (e. g. the knee or shoulder) as the initial involvement in RA is distinctly uncommon. This subgroup of RA is reported to be more severe with worse prognosis [1,2]. However, similar information is not available on Indian RA patients. Therefore, the present study was conducted to determine the initial joint involvement in Indian RA patients. The disease severity and response to drug therapy of this group was then compared with the group where the initial joint involvement was other than the knee or shoulder (predominantly small joints of the hand, feet or several joints simultaneously).

Department of Medicine, Government Medical college, Thiruvanthapuram Introduction: The occurrence of interstitial lung disease with JO1 positivity along with associated raynauds phenonmenon, joint pain, and fever, mechanic hands, with or without muscle weakness is also called antisynthetase syndrome. We are presenting two different cases of interstitial lung disease along with JO1 positivity. First case is of a 33yr old female who presented with dry and scaly lesions over the lateral aspects of both palms and was diagnosed to have mechanic hands. She also had progressive proximal weakness of her four limbs for the past 2 months. Past history of raynauds phenomenon was present. Investigations revealed a very high CPK along with a myopathic pattern in EMG and muscle biopsy evidence of inflammatory polymyositis. ANA was positive with strongly positive antiJO1 antibodies. She had Dypsnea on exertion with PFT showing a restrictive pattern. HRCT showed nonspecific interstitial pneumonitis (NSIP). Second case is of a 55yr old man who presented with 2 month h/o Dypsnea on exertion. There was h/o of inflammatory arthritis of small and large joints with associated fever since 1 month. Investigation revealed raised ESR and CRP. Negative rheumatoid factor and Anticittrulinated peptide antibody, ANA was positive with strong positive anti JO1 antibody. PFT showed a restrictive pattern and HRCT findings were consistent with ILD in the form of NSIP. Both of the patients were started on steroids with good response. Thus JO1 positivity and mechanic hands can be a specific markers for associated ILD.

P65 Spontaneous carotico-cavernous fistula in ehler danlos syndrome Praveen Kumar Yadav, C Jayakumar, Jacob Anthony Department of Medicine, Government Medical college, Thiruvanthapuram

Department of Rheumatology; ISIC Super-specialty hospital, Delhi

Methods: Rheumatoid arthritis patients followed up for at least a year were asked to recall first joint involvement. Fifty consecutive patients seen between Jan 2009 and May 2009 who were able to recall first joint involved were included. Results: The study cohort was of 50 (43 females) patients with a mean age of 46.5 yr (SD + 10.98) at diagnosis. 31 (62%) were seropositive with baseline DAS28 (SD) 5.603 (± 1.07). At 1 year follow-up DAS28 score was 3.015 (± 0.87). Difference by paired t-test was statistically significant (p < 0.001). Eighteen (36%) and 11 (22%) patients reported knee and shoulder joint as the first involvement, respectively. Among the remaining 21 (42%) patients the initially affected joints were other than the knee or shoulder joints (predominantly small joints of the hand, feet or several joints simultaneously). The baseline DAS28 of the patients with knee as the initial involvement (5.825 ± 0.99) was significantly higher than those with initial small joint or multiple joint involvement (5.14 ± 1.03) (p = 0.043). The same was true on comparison of initial shoulder involvement (6.26 + 0.91) with initial small joint or multiple joint involvement (p = 0.006). The shoulder and the knee group patients did not differ significantly between them (p = 0.276). At 1 year there was no significant difference in the disease severity between any of the groups (p value varied from 0.151 to 0.808). When baseline and 1 year follow up DAS28 were compared, patients with the knee and shoulder as the initial affected joints had improved significantly more than those with small joint involvement or multiple joint involvement as the initial manifestation. (Standardized mean difference of knee = 1.98, shoulde r = 1.83, small joints = 1.75)