Pooja Dhaon

Type 1 diabetes and osteoporosis: A review of literature.

With better care and intensive insulin therapy, microvascular complications have reduced and longevity has increased in patients with type 1 diabetes (T1DM). Therefore, there is a need to change the focus from microvascular complications to cardiovascular disease and osteoporosis. Though number of studies from other parts of the world show that patients with T1DM are at increased risk of osteoporosis and fractures, there is a paucity of data from India. A number of factors and mechanisms affecting bone health in patients with T1DM have been proposed. The main defect in genesis of osteoporosis is osteoblastic function, rather than osteoclastic overfunction. Assessment of bone mineral density by dual X-ray absorptiometry and other risk factors for osteoporosis, as a part of diagnostic procedure can help to design tailored treatment plans. A physically active healthy lifestyle, prevention of diabetic complications and adequate calcium and vitamin D supplementation are the mainstay for prevention of osteoporosis. Treatment of osteoporosis is not evidence based but it is proposed to be similar to osteoporosis associated with other conditions. Bisphosphonates are the mainstay for treatment of osteoporosis in patients with T1DM. However, more studies are needed to make definitive guidelines on prevention and treatment of osteoporosis in patients with T1DM.

Oral Methotrexate in split dose weekly versus oral or parenteral Methotrexate once weekly in Rheumatoid Arthritis: a short‐term study

To investigate whether methotrexate (MTX) administered orally to rheumatoid arthritis (RA) patients in split doses at 2–3 days’ interval, would result in equal or better efficacy, tolerability and compliance, without increasing toxicity compared to single weekly dose given orally or parenterally.

Cutaneous polyarteritis nodosa presenting with digital gangrene and breast ulcer.

Dear Editor, Lindberg described cutaneous polyarteritis nodosa (CPAN) in 1931 as a benign form of disorder which affects the small and medium-sized arteries confined to the skin, unlike polyarteritis nodosa which is aggressive and often fatal due to multiorgan involvement. The most common clinical manifestations are subcutaneous nodules, livedo reticularis and ulceration of the lower extremity. Arthralgia, myalgia neuropathy and constitutional features may also be present. The etiology is still unknown, although an immune-mediated mechanism has been postulated with several infectious and noninfectious conditions associated with both initiation and relapse of the disease. We present a case of a patient with severe ischemic loss of both lower limbs and breast ulcer as an unusual initial manifestation of CPAN. A 23-year-old woman presented with a history of high-grade fever for 2 days followed by pain and discoloration of both feet which ended in gangrene in 10 days. She also had an ulcer on her right breast which developed over the last 10 days. There was no history of purpura, Raynaud’s phenomenon, recurrent oral ulcers, hair loss or malar rash. She did not have a bad obstetric history and her menstrual cycle was normal. She was not on oral contraceptive pills or any drugs or had any addictions (tobacco, cocaine, ergot), which would have resulted in the ischemic insult. The general physical examination showed normal vitals. Gangrene was present on both feet extending up to the ankle joint with discoloration extending up to the mid-part of the leg (Fig. 1a). Overlying skin was cold with blister formation. The breast ulcer was approximately 4 cm in size (Fig. 1b). Peripheral pulses, both anterior tibial, posterior tibial and dorsalis pedis, were not palpable and the right radial was weak. All other pulsations were present and there was no bruit over the carotid or renal arteries. The laboratory investigations showed hemoglobin 12.6 g/dL, white blood cell count 9600/lL, platelet 228 000/lL, erythrocyte sedimentation rate 50 mm/h and normal C-reactive protein (CRP). Renal and liver functions were normal. Urine analysis was normal. Blister fluid was sterile. Chest radiographs, two-dimensional echocardiography and abdominal ultrasound were

Prevalence of rheumatic musculoskeletal symptoms in rural and urban areas : a cross-sectional study in northern India

To study the prevalence of rheumatic musculoskeletal symptoms in rural and urban areas of Lucknow.

Rheumatoid nodulosis with extra-articular cyst synovitis

A 50-year-old female patient presented to the Rheumatology Outpatient Departiment with pain involving small and large joints for the last 20 years. On clinical examination she had tenderness of multiple joints along with deformities but no synovitis (Fig. 1a). She also had multiple subcutaneous nodules on both hands and a cystic lesion at the elbow (Fig. 1a, b), which had developed gradually over the last 5 years. Investigations revealed raised erythrocyte sedimentation rate, positive rheumatoid factor (RF), positive anti-cyclic citrullinated peptide (anti-CCP; 170 U/mL) and normal uric acid. High-resolution ultrasound of the cystic lesion at the elbow revealed anechoic collection in the oleacranon bursa with frond like projections with normal elbow joint space (Fig. 1c). Radiograph of both hands showed presence of juxtra-articular osteopenia, joint space narrowing, erosions and deformities (Fig. 1d). Fluid aspirated from the oleacranon bursa was sterile and negative for crystals. An excision biopsy of the oleacranon bursa was performed and its histology showed fibrocollagenous tissue infiltrated with necrotizing granulomas (Fig. 1e–g) with central fibrinoid necrosis.

Performances of Clinical Disease Activity Index (CDAI) and Simplified Disease Activity Index (SDAI) appear to be better than the gold standard Disease Assessment Score (DAS‐28‐CRP) to assess rheumatoid arthritis patients

To compare the performance of Disease Assessment Score of 28 joints – C‐reactive protein (DAS‐28‐CRP), Clinical Disease Activity Index (CDAI) and Simplified Disease Activity Index (SDAI) composite measures to assess status of patients with rheumatoid arthritis (RA) on methotrexate, versus DAS‐28 CRP as the gold standard.

Coombs negative hemolytic anemia with Rheumatoid arthritis - a rare association.

Dear Editor, Rheumatoid arthritis (RA) is complicated with a number of hematological abnormalities. Anemia of chronic disease is the most common association, while autoimmune hemolytic anemia (AIH) is a rare association. There are very few case reports of this association in the literature. We report an RA patient with Coombs negative AIH. A 40-year-old female patient presented to the Rheumatology Department with joint pain of 5 years duration. On examination she was found to have large joint arthritis involving both knees and ankles along with early morning stiffness of more than 2 h. She did not have any history of inflammatory backache or features of lupus. Her investigations revealed low hemoglobin of 80 g/L (Normal-120–160 g/L), white blood cell count of 5.5 9 10/L (Normal-4– 10 9 10/L), platelet count of 163 9 10/L (Normal150–350 9 10/L), erythrocyte sedimentation rate of 40 mm/h (Normal-0–20 mm/h), Creactive protein of 19 nmol/L (Normal-0–8 nmol/L) along with positive rheumatoid factor of 1250 IU/mL, (Normal< 40 IU/mL) and anti-cyclic citrullinated peptide of 198 Relative Unit (RU)/ml, (< 20 RU) in high titers. Her hand radiographs did not show any erosion, but bone scan was suggestive of active arthritis in both knees, elbows, shoulders and ankles. The patient fulfilled the 2010 American College of Rheumatology criteria for RA and she did not have any feature of any other disease. The patient was diagnosed as seropositive RA and was advised as to routine baseline investigations before starting disease modifying drugs. The patient also gave a history of three episodes of jaundice in the last 3 years. Serum bilirubin was 54.3 lmol/L (Normal< 26 lmol/L) with raised unconjugated bilirubin of 45.6 lmol/L (Normal-4.6– 19.5 lmol/L). Aspartate aminotransferase and alanine aminotransferases were 73 IU/L (Normal-0–35 IU/L) and 84 IU/L (Normal-3–36 IU/L). Viral markers were negative. Antinuclear antibody was positive with a speckled pattern with normal complement levels. Anti-double-stranded DNA, anti-LKM-1 (Liver kidney microsomal) antibody and anti-smooth muscle antibodies were negative. Ultrasound examination of the abdomen revealed moderate hepatomegaly and splenomegaly. Red blood indices showed mean corpuscular volume of 98.2 fL (Normal-80–100 fL), mean corpuscular hemoglobin of 30.9 pg/cell (Normal-28– 32 pg/cell), mean corpuscular hemoglobin concentration of 315 g/L (Normal-320–360 g/L), low hematocrit of 27.3% (Normal-36–47%) and high red cell distribution width of 18.3% (Normal-10.8–14.1%). Peripheral blood smear showed moderate anisopoikilocytosis, macrocytosis with microcytic hypochromic red blood cells. The reticulocyte count was high at 10% (Normal-0.5–1.5%), corrected reticulocyte count at 6.18% (Normal-0–1.5%), absolute reticulocyte count at 0.28 9 10/lL (Normal-0.023–0.09 9 10/ lL) and reticulocyte production index was 3.1%. Iron profile showed raised ferritin at 342 lg/L (Normal15–200 lg/L) and normal serum iron at 22 lmol/L (Normal-11–32 lmol/L). Her serum folate and serum vitamin B12 levels were normal. Her lactate dehydrogenase was mildly elevated at 336 U/L (Normal< 195 U/L) while haptoglobin was normal. The clinical and hematological picture was suggestive of hemolytic anemia. Serum haptoglobin was not reduced, possibly due to the presence of inflammation as it is an acute phase reactant. There was no history of any drug intake leading to hemolysis. The direct and indirect Coombs tests were negative. G6PD activity, hemoglobin electrophoresis and bone marrow examination were normal. The patient was given steroids: 1 mg/kg oral prednisolone along with 150 mg azathioprine. The patient responded to treatment with improvement in arthritis, hemoglobin and reduction in reticulocyte count. As steroids were tapered, there was a relapse in hemolysis and the patient was switched over to mycophenolate mofetil from azathioprine. She responded and steroids have been tapered. Thus, even

Arthritis in Stickler syndrome: Inflammatory or degenerative?

Dear Editor, Osteochondrodysplasias are a heterogeneous group collagenopathies with variable clinical picture, radiographic findings and genetic inheritance. Stickler syndrome is the most common collagenopathy which was first recognized by Stickler et al. in a family with ocular, orofacial and musculoskeletal abnormalities. It occurs due to mutations in COL2A1, COL11A1 or COL11A2 genes. Large joint premature osteoarthritis is a feature of this syndrome which occurs in teens and is quiet debilitating. We describe two sisters with Stickler syndrome-like features, who presented with inflammatory arthritis along with positive serological markers and radiographic changes mimicking juvenile idiopathic arthritis. Patient 1 was a 19-year-old woman, the third child of a consanguineous couple, presented with history of joint pains since the last 7 years. She experienced joint pains since the age of 12 involving bilateral metacarpophalangeal joints, wrist, elbow, shoulder and knee along with a morning stiffness of more than 30 min. Gradually she started developing flexion deformities of the hand joints. On examination she was not dysmorphic and had an average stature. She had moderate to severe sensorineural hearing loss since birth, and skeletal abnormalities such as hypertelorism, flat nasal bridge and pectus carinatum. Joint examination showed presence of tenderness and swelling of bilateral wrist joints with presence of flexion deformities (Fig. 1a). The rest of the systemic examination was unremarkable. Patient 1’s sister (Patient 2), aged 17 years had joint pains since the last 2 years, involving bilateral wrist, shoulder and metacarpophalangeal joints along with morning stiffness of more than 30 min. On examination she was not dysmorphic and had an average stature. She had moderate to severe sensorineural hearing loss since birth, bifid uvula and skeletal abnormalities such as hypertelorism, flat nasal bridge and pectus carinatum (Fig. 2a). Joint examination showed presence of tenderness and swelling of some proximal interphalangeal joints, metacarpophalangeal joints and bilateral wrist joints. The rest of the systemic examination was unremarkable. Neither of the patients had ocular findings, mental retardation or any features suggestive of hypermobility. Both the parents and other three siblings were normal and there was no other significant family history. Both the patients had raised erythrocyte sedimentation rate (Patient 1: 105 mm/h, Patient 2: 130 mm/h), positive C-reactive protein and positive rheumatoid factor. Anticyclic citrullinated peptide (anti-CCP) antibody was positive in high titer (Patient 1: 344, Patient 2: 255). Both had positive anti-nuclear antibodies (ANA) with a homogeneous pattern. Radiograph of the hand of Patient 1 showed carpel crowding, periarticular osteopenia, flexor deformities and widening of the metacarpal epiphysis (Fig. 1b). High-resolution ultrasound of the hand showed synovitis of wrists and erosions at the carpal and metacarpal bones. Radiograph of the lumber spine and pelvis showed mild platyspondyly, and flattened with superior-lateral dislocation of the left femoral head (Fig. 1c,d). Radiograph of hand of Patient 2 showed carpel crowding, periarticular osteopenia and erosions (Fig. 2b). High-resolution ultrasound of the hand showed synovitis at wrist joints and erosions in metacarpal bones. Radiograph of the lumber spine showed spondylolysthesis of the L5 vertebra, presence of Schmorl nodes and end plate abnormalities and posterior osteophytes (Fig. 2c,d). Her radiograph of the pelvis was normal. The patients had orofacial abnormalities, auditory abnormalities and mild skeletal abnormalities, along with a positive family history, that is, first-degree relative affected. Thus, they fulfilled criteria for Stickler syndrome. There was variability in phenotype of both the patients which is very well known with Stickler syndrome. Although it is among the most common autosomal dominant connective tissue disorder, there have been reports where the inheritance is autosomal recessive. Camp et al. described a family of Moroccan origin that consisted of four children with Stickler syndrome, six unaffected children, and two unaffected parents