Seroprevalence of Anti-Citrullinated Protein Antibodies (ACPA) in Patients with Rheumatic Diseases other than Rheumatoid Arthritis.

Objective: To evaluate the prevalence of anti- citrullinated protein antibodies (ACPA) in patients with a variety of rheumatic diseases other than rheumatoid arthritis (RA). Methods: 144 cases of rheumatic diseases other than rheumatoid arthritis (RA) over a period of 1 year were recruited after consenting and followed up for 2 years. Their serum samples were tested for ACPA. Results: ACPA seropositivity of 9.03% was observed in rheumatic diseases other than RA. Conclusion: Whether ACPA seropositivity in non-RA rheumatic diseases indicates a false positive result or an overlap RA syndrome is a mystery yet unsolved. Long term follow ups of these patients will be required to understand the course of rheumatic diseases in relation to ACPA..

Anaemia in Newly Diagnosed Patients of Rheumatoid Arthritis and its Correlation with Disease Activity.

Aim: To detect frequency of anemia in patients of Rheumatoid arthritis (RA) and to establish relationship between hemoglobin level and disease activity in RA. Method: Fifty nine patients of RA fulfilling 2010 ACR/EULAR criteria of RA having disease duration less than two years were included in the study. Haemoglobin (Hb) levels were measured. Disease activity was assessed by DAS-28 score. Results: Among 40/59 (67.80%) anemic cases, 22/40 (55%) patients had anaemia of chronic disease (ACD), 11/40 (27.50%) patients had Iron deficiency anemia (IDA), 3/40 (7.5%) patients had vitamin B(12) deficiency, 1/40 (2.50%) patient had folate deficiency and 3/40 (7.50%) patients had combined IDA and vitamin B(12) deficiency. Duration of disease, rheumatoid factor positivity and occurrence of erosive disease were not significantly different among anaemic and nonanaemic patients (p>0.05 for each). Mean ESR (p>0.02) and DAS-28 (p>0.001) were statistically significantly different among anaemic and nonanaemic patients. Haemoglobin level had significant negative correlation with disease activity (DAS28) in RA cases (r -0.5533, p<0.0001). Conclusion: Anemia was seen in higher frequency in RA patients. Haemoglobin had significantly negative correlation with disease activity (DAS 28) in RA. . Conclusion: Anemia was seen in higher frequency in RA patients. Haemoglobin had significantly negative correlation with disease activity (DAS 28) in RA.

Diagnostic Accuracy of Ultrasonography in Detection of Destructive Changes in Small Joints of Hands in Patients of Rheumatoid Arthritis: A Comparison with Magnetic Resonance Imaging.

BACKGROUND: Ultrasonography can be used in early Rheumatoid Arthritis to determine and to follow parameters of joint inflammation, such as effusion, synovitis, and marginal erosion that can be radiologically occult. We therefore planned a study to investigate whether Ultrasonography could provide information on signs of inflammation and destruction in Rheumatoid Arthritis affected finger joints that was not available with Radiography and compared it to the information provided by Magnetic resonance imaging. STUDY DESIGN: Hospital Based Descriptive Study. METHODS: The study included 30 patients fulfilling American College of Rheumatology 2010 criteria of Rheumatoid Arthritis with no erosions present on radiographs of hands. Erosion, Synovial thickening/vascularity, effusion and Tenosynovitis of Flexor tendon sheath / Extensor tendon sheath were assessed on both Ultrasonography and Magnetic resonance imaging. STASTICAL ANALYSIS: Considering Magnetic resonance imaging as gold standard sensitivity, specificity, positive predictive value, negative predictive value and kappa value of Ultrasonography were calculated. Kappa value was calculated by kappa statistics to show agreement between the two modalities. RESULTS: Ultrasonography and Magnetic resonance imaging had near perfect agreement for detecting synovial thickening and vascularity, substantial agreement for detecting effusion, Flexor tendon sheath / Extensor tendon sheath inflammation, and only moderate agreement for detecting erosions in Metacarpophalangeal and Proximal interphalangeal joints. CONCLUSION: The early diagnosis of Rheumatoid Arthritis by Ultrasonography and MRI is very important to early treatment of Rheumatoid Arthritis. Ultrasonography is a reliable method for assessing inflammatory activity and destructive changes in small joints of hand as the Ultrasonographic findings are comparable to those of MRI.

Management of Pregnancy in Lupus Patients.

Systemic lupus erythematosus (SLE) mostly affects young women of reproductive age group. SLE patients may conceive as any normal woman but complication may occur in these patients if the disease is active. Pregnancy in SLE may lead to 1. Aggravation of SLE (Lupus flare) 2. Pre-term delivery, intrauterine growth retardation and foetal loss (in presence of antiphospholipid antibodies) 3. Neonatal lupus especially in presence of Anti-Ro / La antibody. For a successful pregnancy, both from maternal and foetal aspects, disease should be quiescent for at least six months before the conception. Lupus patients with pregnancy require specific management to improve the maternal and fetal outcomes. Many safe drugs are available for the management of pregnancy in SLE.

Pachydermoperiostosis.

A case of Pachydermoperiostosis (PDP) presented to us in rheumatology clinic with complaints of enlargement and broadening of bilateral hands and feet, grade IV digital clubbing, coarsening of facial features, excessive sweating of the palms, soles during summers.

Alkaptonuric Ochronosis.

Alkaptonuria is an autosomal recessive metabolic disorder characterized by joints and spine involvement, ochronosis and presence of homogentisic acid in urine and its deposition in cartilage, intervertebral disc and other connective tissues, leading to disabling arthritis in elderly individual.

Spondyloepihyseal Dysplasia Congenita.

We describe a case of Spondyloepiphyseal Dysplasia Congenita (SEDc) who presented to our rheumatology clinic with complaints of painful swelling of bilateral elbow, knee, distal and proximal interphalangeal joints (DIP and PIP) of hands and small joints of foot. Patient also complained of restriction during extension of bilateral wrist joint and lateral rotation of neck on both sides. He was also unable to walk without support.

Pathogenesis and Clinical Management of Gouty Arthritis.

Gout, the most common of the crystal arthritides is a result of disturbed uric acid metabolism and precipitation of urate crystals in extra cellular space of joints, periarticular tissue, bones and other organs. In the West, gout affects around 1% of adult men over 45 years of age. The estimated incidence being 0.6 to 2.1 per 1000 per year, with a prevalence of 9.5 to 13.5 per 1000 persons of all ages.1 The incidence of gout has been on rise globally; potentially attributable to recent shifts in diet, lifestyle, medical care, and increased longevity.2 Gout is three to four times more common in males than in pre-menopausal females; incidence in women increases after menopause and after the age of 60, approaches that in men.3 This update aims to highlight recent developments in understanding pathogenesis of gout along with current management strategies.

Stiff Person Syndrome.

Stiff-person syndrome or Moersch-Woltmann is a very rare and disabling neurologic disorder characterized by muscle rigidity and episodic spasms involving axial and limb musculature. It is an autoimmune disorder resulting in a malfunction of aminobutyric acid mediated inhibitory networks in the central nervous system. We describe a patient of stiff person syndrome.

Systemic Lupus Erythematosus with Deep Vein Thrombosis and Cutaneous Ulcer.

We are reporting a case of systemic lupus erythematosus (SLE) with left upper limb and lower limb deep vein thrombosis (DVT) due to protein S deficiency which was aggravated by anticoagulants. Oral anticoagulant-induced skin necrosis also developed in this patient. This patient was negative for anti-phospholipid antibodies (APLA). Such a case is rarity where SLE patient without APLA has protein S deficiency.

Peripheral Neuropathy in Systemic Lupus Erythematosus: Clinical and Electrophysiological Properties and their Association with Disease Activity Parameters.

AIM: To study clinical and electrophysiological properties of peripheral neuropathy (PN) in systemic lupus erythematosus (SLE) and their association with disease activity parameters. METHODS: A hospital-based observational study done among 50 SLE patients after informed consent. History and clinical examination including a detailed neurological examination was carried out. Blood and urine investigation were done and modified SLE disease activity index (SLEDAI)-2000 score was calculated. RESULTS: PN was found in 18 out of 50 (36%) SLE cases as defined electrophysiologically, nine had clinical and nine had subclinical neuropathy. On nerve conduction studies (NCS) 17 patients had axonal pattern. There were significant difference for mean ESR in patients with neuropathy (64.17 ± 42.43 mm/1st hour) and without neuropathy (42.34 ± 27.68 mm/1st hour) (P 0.033) and for mean modified SLEDAI-2000 of patients with neuropathy (15.61 ± 10.09) and without neuropathy (6.84 ± 6.16) (P < 0.05). CONCLUSIONS: The study suggests significant association of peripheral neuropathy in SLE patients with ESR, modified SLEDAI-2000, pyuria, pleurisy and leucopenia.

Familial juvenile hyperuricemic nephropathy 1 (FJHN1).

Familial juvenile hyperuricemic nephropathy 1 (FJHN1) is an autosomal dominant disorder characterized by decreased urinary excretion of urate and hyperuricemia, followed by the development of chronic interstitial nephritis most often leading to progressive renal failure and death in middle age. We report a case of FJHN1 presenting as chronic tophaceous gout, hypertension, renal failure and a family history suggestive of autosomal dominant inheritance, for its rarity.

Estimation of vitamin D levels in rheumatoid arthritis patients and its correlation with the disease activity.

AIM: To detect level of serum vitamin D in patients of Rheumatoid arthritis (RA) and to establish relationship between serum vitamin D level and disease activity in RA. METHOD: Eighty patients of RA fulfilling 1987 revised criteria of the American College of Rheumatology (ACR) of RA classification and eighty healthy controls were included in the study. 25 (OH) vitamin D levels were measured. Disease activity was assessed by DAS-28 score. RESULTS: Ninety percent of RA patients were either vitamin D deficient or insufficient while only seventy percent of healthy controls were either vitamin D deficient or insufficient(p=0.007). Mean serum vitamin D levels of RA patients was significantly low compared to healthy controls (p=0.009). Thirty-one patients had high disease activity (DAS-28 score >5.1, group A), 32 patients had moderate disease activity (DAS 28 score 3.2-5.1, group B) and 17 patients had low disease activity (DAS-28 score <3.2, group C). Vitamin D levels in high disease activity group was significantly low compared to vitamin D level in patients with low and moderate disease activity (p<.001) and vitamin D level had significant negative correlation with DAS28 score (r=-0.604, p<0.001). CONCLUSION: Serum vitamin D levels were significantly low in RA patients than in healthy controls. Vitamin D deficiency was seen in significantly higher numbers of patients and vitamin D had negative correlation with disease activity in RA.

Methaemoglobinaemia as a result of nitrite poisoning.

Methaemoglobinaemia due to nitrite poisoning is rare. Awareness of this condition in the cyanosed patient not responding to oxygenation and timely administration of methylene blue may be life saving. We report a case of methaemoglobinaemia as a result of sodium nitrite poisoning.

Anti-nucleosome antibodies in patients with systemic lupus erythematosus: potential utility as a diagnostic tool and disease activity marker and its comparison with anti-dsDNA antibody.

OBJECTIVE: To compare the utility of anti-nucleosome antibodies and anti-dsDNA antibodies in diagnosis of Systemic Lupus Erythematosus (SLE) and as a marker of disease activity. METHODS: This is a hospital based observational study among 40 (37 females and 3 males) selected cases of SLE (> or = 4 ACR criteria) and 80 control. 40 cases of other systemic autoimmune disease (SAD) [e g. 29 cases of Rheumatoid arthritis, 4 cases of Systemic sclerosis/scleroderma, 4 cases of Sjögren syndrome, 3 cases of MCTD and 40 Healthy blood were taken as control. From each patient venous blood samples were collected and submitted for anti-nucleosome and anti-dsDNA antibodies assay by enzyme linked immunosorbent assay (ELISA). RESULTS: Anti-nucleosome antibodies were positive in 19 (47.5%) SLE, 02 (05%) other SAD and none of the healthy persons. Anti dsDNA antibodies were positive in 15 (37.5%) SLE patients, 07 (17.5%) other SAD and 01(2.5%) healthy persons. For diagnosis of SLE, sensitivity of anti-ds DNA and anti-nucleosome antibody was found to be 37.5% and 47.50% respectively. The specificity of anti-nucleosome was 100% and that of anti-dsDNA was 97.50%. So, anti-nucleosome antibody test is more specific and more sensitive for diagnosis of SLE than anti-dsDNA. When SLE cases were compared with SAD, sensitivity of anti-dsDNA and anti-nucleosome antibody, for diagnosis of SLE, found to be 37.50% and 47.50% respectively but the specificity of anti-nucleosome was 95% and that of anti-dsDNA was 82.50%. Both antibodies show positive correlation with SLEDAI score .The correlation coefficient was stronger for anti-dsDNA antibodies (r = +0.550, P = < .001) than anti-nucleosome antibodies (r = +0.332, P = < .05) CONCLUSIONS: Anti-nucleosome antibodies show higher positivity than anti-dsDNA antibodies among SLE than other SAD and healthy population. Anti-nucleosome antibodies are more sensitive and specific for the diagnosis of SLE than anti-dsDNA antibodies. Anti-nucleosome and anti-dsDNA both show positive correlation with SLEDAI. But anti-dsDNA antibodies show stronger correlation with SLEDAI than anti-nucleosome. So, anti-nucleosome antibodies can be used as an additional marker for diagnosis of SLE and SLE disease activity.

Raynaud's phenomenon.

Raynauds phenomena (RP) is a commonly encountered clinical manifestation which may be primary or secondary to underlying disease. There is imbalance between vasoconstricting and vasodilating factors. Physical examination, nailfold capillaroscopy and immunological tests can differentiate primary from secondary RP. Treatment involves prevention of RP so that permanent ischemic damage i.e. gangrene can be avoided. Avoidance of exposure to cold, emotional stress and certain drugs is mandatory and if attacks are occurring then vasodilators, prostaglandin analogues, anticoagulants and antiplatelet drugs may be added. An attempt has been made to guide the clinician to diagnose and treat a patient of RP through this article.

Pachydermoperiostosis with myelofibrosis and empty sella.

A case of pachydermoperiostosis presented to us in rheumatology clinic with complaints of pain and swelling in knee joints unresponsive to treatment, characteristic facial features, grade four clubbing of nails and broadening of distal parts of extremities. He also complained of fatiguability which was due to anemia. The natural history of the disease was reviewed and investigated.

Antiphospholipid antibody syndrome.

The 2006 International Consensus Statement on an Update of the Classification Criteria for Definite Antiphospholipid Syndrome has increased the time between the two laboratory studies required for diagnosis from 6 to 12 weeks. Antibody to beta2 glycoprotein 1 has been included as a criterion. Various non-criteria diagnostic clues such as livedo reticularis, heart valve disease, thrombocytopenia, renal thrombotic microangiopathy, neurological manifestations, non-criteria antibodies (IgA aCL, IgA anti-beta2 glycoprotein I) and some research laboratory-identified antibodies (antiphosphatidylserine antibodies, antiphosphatidylethanolamine antibodies, antibodies against prothrombin alone and antibodies to the phosphatidylserine-prothrombin complex) have been recognised. New concepts of pathogenesis now implicate complement activation and participation of the innate immune system upstream to thrombosis. Warfarin remains the treatment of choice for patients who have suffered thrombosis, but antiplatelet agents and heparin are other options. Target INR is 2.0-3.0. The other drugs which are used in resistant cases are: rituximab, hydroxychloroquine, thrombin inhibitors and statins.