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A 22-year-old lady presented to you with frequent fatigue, joint pain and weight loss. On physical examination noted that she has malar rash and oral ulcers. A diagnosis of systemic lupus erythematosus Which of the following statement is INCORRECT regarding SLE? A. It mainly affects women in high estrogen period B. It is more common in female C. Polyarthritis is the commonest symptom of SLE D. Antinuclear antibody is highly sensitive towards SLE E. Anti-Smith antibody is highly sensitive towards SLE

A 22-year-old lady presented to you with frequent fatigue, joint pain and weight loss. On physical examination noted that she has malar rash and oral ulcers. A diagnosis of systemic lupus erythematosus Which of the following statement is INCORRECT regarding SLE? A. It mainly affects women in high estrogen period B. It is more common in female C. Polyarthritis is the commonest symptom of SLE D. Antinuclear antibody is highly sensitive towards SLE E. Anti-Smith antibody is highly sensitive towards SLE

Correct Answer Is C This patient has presented with signs and symptoms of rheumatoid arthritis. The criteria to diagnose rheumatoid arthritis include the following: – Symptoms duration more than 6 weeks. – Early morning stiffness for more than 1 hour. – Arthritis in three or more joints. – Bilateral compression tenderness of the metatarsophalangeal joints. – Symmetry of the areas affected. – Rheumatoid factor, Anti-CCP antibody positivity. The role of non-steroidal anti-inflammatory drugs to reduce the pain and inflammation is well known in patients with rheumatoid arthritis. However, they do not prevent the joint damage and are associated with many side effects. Standard initial therapy with disease-modifying anti-rheumatic drugs helps to prevent damage to the joint in any patient with the rheumatoid arthritis. Methotrexate is the drug of choice as initial monotherapy. If disease remission is not achieved, increase the dose of methotrexate or consider combination therapy with other agents like sulfasalazine and hydroxychloroquine.

A 32-year-old woman presented with 3 month history of joint pain in both hands and stiffness lasting for hours once she wakes up in the morning every day. She also reported disturbed sleep due to the nocturnal pain. On examination, you notice swelling around metacarpophalangeal (MCP) and proximal interphalangeal (PIP) joint. Which ONE of the following is the drug of choice to reduce the synovial inflammation and prevent joint damage? A. Fish oil B. Naproxen C. Methotrexate D. Sulfasalazine E. Hydroxychloroquine

Correct Answer Is A She was diagnosed as systemic lupus erythematous (SLE) and treated with topical ointments, sunscreen lotion, oral prednisolone (for autoimmune hemolytic anemia) and hydroxychloroquine. Lupus (Latin for wolf) derives its name from the classical butterfly rash which mimics ‘the bite or scratch of a wolf’. Malar rash (butterfly rash), seen in 46–65% of lupus patients, is highly specific for the diagnosis of SLE. The rash may be raised or flat, scaly, non-pruritic and characteristically spares the nasolabial fold (differentiates from dermatomyositis). Common differential diagnoses include melasma (gray brown patches, common in pregnancy), dermatomyositis, lupus pernio and lupus vulgaris (indurated and nodular lesions), pellagra (symmetric keratotic areas on face which always affects body also), seborrheic dermatitis (also on other areas of face), rosacea (erythema, papule or pustules and telengiectasias on nose and medial cheeks, associated ocular features) and sunburn (significant pruritus and temporal association with sun exposure.

A 25-year-old married lady presented with increased hair loss, painless oral ulcers and rash over the face. She also had arthralgias involving small and large joints with early morning stiffness for 8 months. Physical examination revealed nonscarring alopecia, fixed erythematous maculopapular rash with hyperpigmen- tation over the cheeks and nasal bridge sparing nasolabial fold (Figure 1A). Multiple oral ulcers were also noted over the hard palate (Figure 1B). Investigations were notable for autoimmune hemolytic anemia (direct coombs test positive), leucopenia, anti- nuclear antibody positivity by indirect immunofluorescence method (dilution 1:160, 2þ intensity, pattern homogenous) and low complement (C3 and C4). Discuss and what is your diagnosis? Figure 1. (A) Maculopapular rash over the malar area in the shape of a butterfly, which is sparing the nasolabial folds (marked by black lines) and (B) multiple oral ulcers on the hard palate (black arrows). A. Systemic lupus erythematosus B. Pellagra C. Lupus Pernio D. Dermatomyositis E. Melasma

A

Correct Answer Is A The clinical picture of symmetrical swelling and tenderness of the metacarpophalangeal (MCP) and wrist joints lasting longer than 6 weeks strongly suggest rheumatoid arthritis. Rheumatoid factor, an immunoglobulin directed against the Fc portion of IgG, is positive in about two-thirds of cases and may be present early in the disease. The history of lethargy or fatigue is a common prodrome of RA. The inflammatory joint changes on examination are not consistent with chronic fatigue syndrome; furthermore, patients with CFS typically report fatigue existing for many years. The MCP-wrist distribution of joint symptoms makes osteoarthritis very unlikely. The x-ray changes described are characteristic of RA, but would occur later in the course of the disease. Although arthritis can occasionally be a manifestation of hematologic malignancies and, rarely, other malignancies, the only indicated screening would be a complete history and physical examination along with a CBC.

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A 40-year-old woman complains of 7 weeks of pain and swelling in both wrists and knees. She has several months of fatigue. After a period of rest, resistance to movement is more striking. On examination, the metacarpophalangeal joints and wrists are warm and tender. There are no other joint abnormalities. There is no alopecia, photosensitivity, kidney disease, or rash. Which of the following is correct? A. The clinical picture suggests early rheumatoid arthritis, and a rheumatoid factor should be obtained. B. The prodrome of lethargy suggests chronic fatigue syndrome. C. Lack of systemic symptoms suggests osteoarthritis. D. X-rays of the hand are likely to show joint space narrowing and erosion. E. An aggressive search for occult malignancy is indicated

Correct Answer Is A The ‘bumps’ shown in the photo are rheumatoid nodulosis. They are the most common cutaneous manifestation of rheumatoid arthritis (RA). The presence of subcutaneous rheumatoid nodules is associated with an increased risk of cardiovascular disease, as well as cardiovascular, respiratory, and all-cause mortality. The nodules are usually non-tender, and treatment is initiated only for cosmetic or functional reasons. Local glucocorticoid injections have been shown to decrease the size of subcutaneous rheumatoid nodules. Treatment of RA with methotrexate and other DMARDs can increase the frequency and number of rheumatoid nodules—a condition known as accelerated nodulosis. Discontinuing methotrexate may improve nodulosis, but this may enhance the long-term progression of joint destruction in RA. Topical steroids are used for ophthalmic manifestations of inflammatory conditions but have not been shown to be effective for treating rheumatoid nodulosis. Using NSAIDs may improve the pain but does not reduce the size and number of nodules. Surgery is reserved for patients with neurologic dysfunction or significantly decreased functionality.

A 55-year-old mechanic presented with non-painful, red bumps on his hand for several years as shown below. They lasted for a month and resolved spontaneously. He had underlying arthralgias in multiple joints such as bilateral hands, hips, and knees. During one episode, he was prescribed an oral steroid that seemed to relieve his symptoms. The nodules were firm and varied in size. There was no skin erosion, signs of infection, neurologic dysfunction, or decreased range of motion. The patient requested treatment because the nodules were interfering with his work. Which one of the following therapies should have been offered? A. Intralesional steroid injection B. Topical steroids C. Nonsteroidal anti-inflammatory drugs D. Surgery E. Disease-modifying anti-rheumatic drugs (DMARDs)

Correct Answer Is A Systemic sclerosis is generally subdivided into localized and diffuse cutaneous subtypes.These two subsets are principally distinguished by the extent of skin sclerosis and by the forms of organ involvement. Patients with localized scleroderma typically have skin sclerosis restricted to the hands and, to a lesser extent, to the face and neck. They also have prominent vascular manifestations and may suffer from the CREST syndrome (Calcinosis cutis, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia). The presence of anti-centromere antibodies is usually associated with localized scleroderma in 90% of the cases. Only 5% of patients with diffuse systemic sclerosis have positive anti-centromere antibodies. So anti-centromere antibodies are helpful in making diagnosis of CREST syndrome. Anti-DNA topoisomerase I (Scl-70) antibodies are associated with diffuse cutaneous systemic sclerosis. All other given options are not helpful in making diagnosis of scleroderma.

A 34-year-old woman presented with swallowing difficulties, calcinosis and Raynaud’s phenomenon for the last 3 years. Which of the following antibody is most likely to help in establishing the diagnosis? A. Anti-centromere antibodies B. Anti-scl-70 C. Antinuclear antibody D. Rheumatoid factor E. Double stranded DNA

Correct Answer Is C The male is suffering from Cystic Fibrosis and would benefit from having a sweat chloride test. Cystic fibrosis is an autosomal recessive disorder resulting in a defect in an ion channel protein which affects the normal transport of chloride ions, leading to a decreased sodium and water transfer, thus causing viscid secretions. Clinical features include malaise, failure to thrive, chronic respiratory problems, malabsorption, infertility in males (atrophy of vas deferens) and pancreatic insufficiency.

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