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A case of arthritis with digital gangrene
Indian Journal of Rheumatology 2012 June Volume 7, Number 2; pp. 123e124
Letter to the Editor
A case of arthritis with digital gangrene Biswadip Ghosha,*, Shyamashis Dasb, Atanu Palb, Suchandra Rayc, Sanjay Dhard, Alakendu Ghoshe Keywords: Raynaud’s phenomenon, Gangrene, Sjogren’s syndrome
Dear Editor, Primary Sjogren’s syndrome is a multisystem disease and its clinical manifestations are divided into the exocrine gland features (‘sicca complex’) and the extraglandular disease features. Gangrene is a rare presenting feature of it, usually resulting from vasculitis or secondary anti-phospholipid antibody syndrome. Here we report a case of primary Sjogren’s syndrome with gangrene due to a rare cause.
CASE REPORT A 40-year-old housewife presented with pain and swelling of small and large joints of upper and lower limbs for one year. She also gave history suggestive of triphasic Raynaud’s phenomenon during winter season for past two years. She complained of recent onset of burning sensation of both feet. On further questioning she informed that she was having dryness of mouth and grittiness in eyes since the onset of her illness. There was no history of skin tightness, skin rash, photosensitivity, oral ulcer, fever, proximal or distal muscle weakness or exertional dyspnea. She was non-diabetic, non-hypertensive and non-smoker. Examination revealed puffy hands with tender and swollen MCP, PIP and wrist joints. There were no finger tip ulcers or scars. Neurologic, Respiratory and cardiovascular systems were within normal limits. Investigations showed: Hb 11.5 g/dL, WBC 9600/cmm, platelets 250,000/cmm; urine routine examination e within normal limits; Rheumatoid factor e negative; ANA e positive with 1:640 titer with speckled pattern; anti-U1RNP e negative; anti SCL 70 e negative; anti-SSA e strongly
a
positive and anti-SSB e negative. Blood borne virus screening, namely HBsAg, Anti-HCV and HIV, was negative. A pulmonary function test showed mild restrictive pattern, though following HRCT thorax did not reveal any evidence of interstitial lung disease. She was started with hydroxychloroquine, low dose prednisolone and nifedipine and asked for follow up. She returned after one month with newly developed gangrene affecting tip of left index, right middle fingers and 2nd toe of right foot (Fig. 1). It was of acute onset but gradually progressing proximally. She did not have any fever, weight loss or skin rashes. All peripheral pulses were palpable. An arterial Doppler of four limbs did not show any significant abnormalities. Her echocardiography was normal. Lupus anticoagulant and anti-cardiolipin antibodies (both IgM and IgG) were negative. Her serum homocysteine level and lipid profile were within normal limits. Test for cryoglubilin was also negative. A nerve conduction study showed mild distal sensory neuropathy of both lower limbs with features of bilateral S1 radiculopathy. A sural nerve biopsy showed mild nerve fiber degeneration without any evidence of inflammation. Schirmer’s test was 2 mm (in right eye) and 3 mm (in left eye) in 5 min. Tear breakup time done with fluorescin dye was less than 5 s in both eyes. A biopsy of minor salivary glands from lip showed chronic inflammation of glands with dense lymphocytic and plasma cell infiltration with focal area of hyalinization (Fig. 2). There was more than one focus of lymphocyte aggregate per 4 sq mm of salivary tissue (i.e. grade 4 inflammation).1 So, the final diagnosis is primary Sjogren’s syndrome with gangrene attributed to Raynaud’s phenomenon.
Assistant Professor, bClinical Tutor, Department of Rheumatology and Clinical Immunology, cAssociate Professor, Department of Pathology, Research Officer, eProfessor and Head, Department of Rheumatology and Clinical Immunology, IPGME&R and SSKM Hospital, 244, AJC Bose Road, Kolkata, India. * Corresponding author. Tel.: +91 9433272612, email: [email protected] Received: 11.11.2011; Revised: 10.4.2012; Accepted: 11.4.2012; Available online: 17.5.2012 Copyright © 2012, Indian Rheumatology Association. All rights reserved. d
doi:10.1016/j.injr.2012.04.010
124
Indian Journal of Rheumatology 2012 June; Vol. 7, No. 2
Ghosh et al.
Fig. 1 Digital gangrene in a 40-year-old lady. Fig. 2 Minor salivary gland shows chronic inflammatory cells.
She was treated with tear substitute, sustained release nifedipine, Tadalafil (for Raynaud’s phenomenon), aspirin, HCQ and low dose prednisolone along with antibiotics for secondary infection of the gangrene. After two months of follow up, gangrene became dry with clear demarcation. Her sicca symptoms and arthritis also improved.
dryness or significant organ system involvement. Primary SS presenting with multiple digital gangrene is a rare incidence. Gangrene due to Raynaud’s phenomenon is even rarer in primary SS. Because of the rarity of the incidence we are reporting this case.
DISCUSSION REFERENCES Raynaud’s phenomenon (RP) is not uncommon in primary Sjogren’s syndrome (SS), occurrence ranging from 13 to 66%.2 It is often associated with non-erosive arthritis, sometimes precedes the onset of xerostomia (45%). Compared with SS patients without RP, those with RP showed a higher prevalence of anti-nuclear antibodies.3 Gangrene in Sjogren’s Syndrome may occur due to vasculitis, associated anti-phospholipid antibody syndrome or Raynaud’s phenomenon. Occurrence of vasculitis in primary SS affecting small and medium size vessels is around 15%.4 Palpable purpura, skin ulcers, neuropathy and gangrene are major manifestation of vasculitis in SS. But, in the present case histopathology of sural nerve did not show any evidence of vasculitis. Secondary anti-phospholipid antibody syndrome had also been excluded in this case. Raynaud’s phenomenon as a cause of gangrene in SS is rare. In a series of 320 patients of primary SS, only one patient had distal gangrene due to RP.3 Early diagnosis of primary Sjogren’s syndrome is not common in India,5,6 unless patients present with severe
1. Chisholm DM, Mason DK. Labial salivary gland biopsy in Sjögren’s disease. J Clin Pathol. 1968;21:656e660. 2. Skopouli FN, Talal A, Galanopoulou V, et al. Raynaud’s phenomenon in primary Sjögren’s syndrome. J Rheumatol. 1990;17:618. 3. Garcia-Carrasco M, Siso A, Ramos-Casals M, et al. Raynaud’s phenomenon in primary Sjögren’s syndrome: prevalence and clinical characteristics in a series of 320 patients. J Rheumatol. 2002;29:726. 4. Tsokos M, Lazarou SA, Moutsopoulos HM. Vasculitis in primary Sjögren’s syndrome: histologic classification and clinical presentation. Am J Clin Pathol. 1987;88:26. 5. Porkodi R, Rukmangatharajan S, Kanakarani P, et al. Primary Sjögren’s syndrome e clinical and immunological features. J Ind Rheumatol Assoc. 2003;11:63e65. 6. Misra R, Hissaria P, Tandon V, et al. Primary Sjögrens syndrome: rarity in India. J Assoc Phys India. 2003;51: 859e862.
Letter to the Editor
A case of arthritis with digital gangrene Biswadip Ghosha,*, Shyamashis Dasb, Atanu Palb, Suchandra Rayc, Sanjay Dhard, Alakendu Ghoshe Keywords: Raynaud’s phenomenon, Gangrene, Sjogren’s syndrome
Dear Editor, Primary Sjogren’s syndrome is a multisystem disease and its clinical manifestations are divided into the exocrine gland features (‘sicca complex’) and the extraglandular disease features. Gangrene is a rare presenting feature of it, usually resulting from vasculitis or secondary anti-phospholipid antibody syndrome. Here we report a case of primary Sjogren’s syndrome with gangrene due to a rare cause.
CASE REPORT A 40-year-old housewife presented with pain and swelling of small and large joints of upper and lower limbs for one year. She also gave history suggestive of triphasic Raynaud’s phenomenon during winter season for past two years. She complained of recent onset of burning sensation of both feet. On further questioning she informed that she was having dryness of mouth and grittiness in eyes since the onset of her illness. There was no history of skin tightness, skin rash, photosensitivity, oral ulcer, fever, proximal or distal muscle weakness or exertional dyspnea. She was non-diabetic, non-hypertensive and non-smoker. Examination revealed puffy hands with tender and swollen MCP, PIP and wrist joints. There were no finger tip ulcers or scars. Neurologic, Respiratory and cardiovascular systems were within normal limits. Investigations showed: Hb 11.5 g/dL, WBC 9600/cmm, platelets 250,000/cmm; urine routine examination e within normal limits; Rheumatoid factor e negative; ANA e positive with 1:640 titer with speckled pattern; anti-U1RNP e negative; anti SCL 70 e negative; anti-SSA e strongly
a
positive and anti-SSB e negative. Blood borne virus screening, namely HBsAg, Anti-HCV and HIV, was negative. A pulmonary function test showed mild restrictive pattern, though following HRCT thorax did not reveal any evidence of interstitial lung disease. She was started with hydroxychloroquine, low dose prednisolone and nifedipine and asked for follow up. She returned after one month with newly developed gangrene affecting tip of left index, right middle fingers and 2nd toe of right foot (Fig. 1). It was of acute onset but gradually progressing proximally. She did not have any fever, weight loss or skin rashes. All peripheral pulses were palpable. An arterial Doppler of four limbs did not show any significant abnormalities. Her echocardiography was normal. Lupus anticoagulant and anti-cardiolipin antibodies (both IgM and IgG) were negative. Her serum homocysteine level and lipid profile were within normal limits. Test for cryoglubilin was also negative. A nerve conduction study showed mild distal sensory neuropathy of both lower limbs with features of bilateral S1 radiculopathy. A sural nerve biopsy showed mild nerve fiber degeneration without any evidence of inflammation. Schirmer’s test was 2 mm (in right eye) and 3 mm (in left eye) in 5 min. Tear breakup time done with fluorescin dye was less than 5 s in both eyes. A biopsy of minor salivary glands from lip showed chronic inflammation of glands with dense lymphocytic and plasma cell infiltration with focal area of hyalinization (Fig. 2). There was more than one focus of lymphocyte aggregate per 4 sq mm of salivary tissue (i.e. grade 4 inflammation).1 So, the final diagnosis is primary Sjogren’s syndrome with gangrene attributed to Raynaud’s phenomenon.
Assistant Professor, bClinical Tutor, Department of Rheumatology and Clinical Immunology, cAssociate Professor, Department of Pathology, Research Officer, eProfessor and Head, Department of Rheumatology and Clinical Immunology, IPGME&R and SSKM Hospital, 244, AJC Bose Road, Kolkata, India. * Corresponding author. Tel.: +91 9433272612, email: [email protected] Received: 11.11.2011; Revised: 10.4.2012; Accepted: 11.4.2012; Available online: 17.5.2012 Copyright © 2012, Indian Rheumatology Association. All rights reserved. d
doi:10.1016/j.injr.2012.04.010
124
Indian Journal of Rheumatology 2012 June; Vol. 7, No. 2
Ghosh et al.
Fig. 1 Digital gangrene in a 40-year-old lady. Fig. 2 Minor salivary gland shows chronic inflammatory cells.
She was treated with tear substitute, sustained release nifedipine, Tadalafil (for Raynaud’s phenomenon), aspirin, HCQ and low dose prednisolone along with antibiotics for secondary infection of the gangrene. After two months of follow up, gangrene became dry with clear demarcation. Her sicca symptoms and arthritis also improved.
dryness or significant organ system involvement. Primary SS presenting with multiple digital gangrene is a rare incidence. Gangrene due to Raynaud’s phenomenon is even rarer in primary SS. Because of the rarity of the incidence we are reporting this case.
DISCUSSION REFERENCES Raynaud’s phenomenon (RP) is not uncommon in primary Sjogren’s syndrome (SS), occurrence ranging from 13 to 66%.2 It is often associated with non-erosive arthritis, sometimes precedes the onset of xerostomia (45%). Compared with SS patients without RP, those with RP showed a higher prevalence of anti-nuclear antibodies.3 Gangrene in Sjogren’s Syndrome may occur due to vasculitis, associated anti-phospholipid antibody syndrome or Raynaud’s phenomenon. Occurrence of vasculitis in primary SS affecting small and medium size vessels is around 15%.4 Palpable purpura, skin ulcers, neuropathy and gangrene are major manifestation of vasculitis in SS. But, in the present case histopathology of sural nerve did not show any evidence of vasculitis. Secondary anti-phospholipid antibody syndrome had also been excluded in this case. Raynaud’s phenomenon as a cause of gangrene in SS is rare. In a series of 320 patients of primary SS, only one patient had distal gangrene due to RP.3 Early diagnosis of primary Sjogren’s syndrome is not common in India,5,6 unless patients present with severe
1. Chisholm DM, Mason DK. Labial salivary gland biopsy in Sjögren’s disease. J Clin Pathol. 1968;21:656e660. 2. Skopouli FN, Talal A, Galanopoulou V, et al. Raynaud’s phenomenon in primary Sjögren’s syndrome. J Rheumatol. 1990;17:618. 3. Garcia-Carrasco M, Siso A, Ramos-Casals M, et al. Raynaud’s phenomenon in primary Sjögren’s syndrome: prevalence and clinical characteristics in a series of 320 patients. J Rheumatol. 2002;29:726. 4. Tsokos M, Lazarou SA, Moutsopoulos HM. Vasculitis in primary Sjögren’s syndrome: histologic classification and clinical presentation. Am J Clin Pathol. 1987;88:26. 5. Porkodi R, Rukmangatharajan S, Kanakarani P, et al. Primary Sjögren’s syndrome e clinical and immunological features. J Ind Rheumatol Assoc. 2003;11:63e65. 6. Misra R, Hissaria P, Tandon V, et al. Primary Sjögrens syndrome: rarity in India. J Assoc Phys India. 2003;51: 859e862.