Rheumatoid arthritis is a polyarthritis that results in symmetrical pain and swelling of the affected joints (also at rest). It particularly affects the metacarpophalangeal joints (MCPJs) and proximal interphalangeal joints (PIPJs), not the distal interphalangeal joints (DIPs).
Ulcerative colitis and IBD are associated with seronegative arthritides, not RA. The condition can also cause various extra-articular manifestations such as ocular symptoms, rheumatoid nodules and pulmonary fibrosis. Scleritis, episcleritis and keratoconjunctivitis sicca are more common than uveitis. Early intervention with disease-modifying antirheumatic drugs (DMARDs) plays a decisive role in successful treatment.

Polymyositis is an inflammatory disorder causing symmetrical, proximal muscle weakness. It is thought to be a T-cell mediated cytotoxic process directed against muscle fibres. It may be idiopathic or associated with connective tissue disorders. It may also be associated with malignancy for example small cell lung carcinoma.
Dermatomyositis is a variant of the disease where skin manifestations are prominent, for example a purple (heliotrope) rash on the cheeks and eyelids.
It typically affects middle-aged, female: male 3:1. Features include proximal muscle weakness +/- tenderness, Raynaud’s phenomenon, respiratory muscle weakness, interstitial lung disease: e.g. fibrosing alveolitis or organising pneumonia, dysphagia, dysphonia. Investigations: elevated creatine kinase, other muscle enzymes (lactate dehydrogenase (LD), aldolase, AST and ALT) are also elevated in 85-95% of patients, EMG, muscle biopsy. Anti-Jo-1 antibodies are seen in pattern of disease associated with lung involvement, Raynaud’s and fever. Interstitial lung disease plays a major role in morbidity and mortality in patients with polymyositis and is considered a major risk factor for premature death in patients with myositis.

Prednisolone although has many undesirable side effects it may be considered relatively safe compared to the drugs that are provided here. Prolonged treatment with sulphasalazine may depress semen quality and cause irreversible infertility. Methotrexate and leflunomide both inhibit purine/pyrimidine synthesis (the former by inhibiting folate metabolism) and are contraindicated in pregnancy or while trying to conceive. In males, a temporary or permanent decrease in sperm count may occur with cyclophosphamide. Because the recovery of fertility after cyclophosphamide therapy is variable, sperm banking should be considered before treatment is begun.

Anti-TNF-α therapy is effective for patients with arthritis but it can oftentimes lead to the reactivation of latent TB. Hence it should be used with great caution in patients with a past history of TB or current infection.

Polymyalgia rheumatica, a syndrome characterized by proximal muscle pain and stiffness in older persons, generally is treated with prednisone. Dosages of 15 to 25 mg of prednisone per day can reduce inflammation considerably, although many patients relapse when therapy is tapered. Long-term (18 to 36 months) steroid treatment has been recommended by several studies, but this can result in multiple side effects, including osteoporosis, hypertension, cataracts, and hyperglycaemia. Methotrexate has been used to reduce inflammation in rheumatoid arthritis, systemic vasculitis, and giant cell arteritis, and in some studies has been combined with prednisone to treat polymyalgia rheumatica, decreasing the duration of treatment.

Paget’s accelerates the remodelling process with old bone breaking down more quickly which disrupts the normal growth process. New bone development eventually adjusts to the faster pace and speeds up on its own. The pace is too fast for healthy bone growth, and the regrowth ends up softer and weaker than normal. The effect of this accelerated process causes bone pain, fractures, and deformities. Since osteosarcoma is a type of bone cancer linked to abnormal bone growth, this makes it a rare but possible complication of Paget’s disease although the chances of developing osteosarcoma are minimal. Fractures are painful and restrict movement whereas this patients pain is gradually developing. Pain associated with osteoarthritis is usually mild to moderate and worsens as the day progresses.

Pseudogout is a paroxysmal joint inflammation due to calcium pyrophosphate crystal deposition (calcium pyrophosphate dihydrate). Aetiology includes mostly idiopathic (primary form) and secondary form occurring as a result of joint trauma, familial chondrocalcinosis, hyperparathyroidism, hemochromatosis, gout, hypophosphatemia. Clinical presentation: Often asymptomatic.
Acute (pseudogout attack): monoarthritis (rarely oligoarthritis), mostly affecting the knees and other large joints (e.g., hips, wrists, and ankles). It may become chronic (can affect multiple joints). Osteoarthritis with CPPD (most common form of symptomatic CPPD): progressive joint degeneration with episodes of acute inflammatory arthritis typical of pseudogout attacks. Arthrocentesis should be performed, especially in acute cases. Polarized light microscopy: detection of rhomboid-shaped, positively birefringent CPPD crystals. Synovial fluid findings: 10,000-50,000 WBCs/μL with > 90% neutrophils. X-ray findings: cartilage calcification of the affected joint (chondrocalcinosis). Fibrocartilage (meniscus, annulus fibrosus of intervertebral disc) and hyaline cartilage (joint cartilage) may be affected.

In rheumatoid arthritis (RA), clinical symptoms of joint stiffness, pain, and functional disability are commonly most severe in the early morning. These symptoms closely follow the circadian rhythm of the pro-inflammatory cytokine, interleukin (IL)-6. In RA, the increase in nocturnal anti-inflammatory cortisol secretion is insufficient to suppress ongoing inflammation, resulting in the morning symptoms characteristic of RA. Established diagnostic criteria for RA include prolonged morning stiffness that could last up to an hour. Loss of joint mobility, pain, malaise and swelling of finger joints are features that are not specific to rheumatoid arthritis, and are found in many other conditions.

Ankylosing spondylitis (spondylarthritis) is a chronic inflammatory disease of the axial skeleton that leads to partial or even complete fusion and rigidity of the spine. Males are disproportionately affected and upwards of 90% of patients are positive for the HLA-B27 genotype, which predisposes to the disease. The most characteristic early finding is pain and stiffness in the neck and lower back, caused by inflammation of the vertebral column and the sacroiliac joints. The pain typically improves with activity and is especially prominent at night. Other articular findings include tenderness to percussion and displacement of the sacroiliac joints (Mennell’s sign), as well as limited spine mobility, which can progress to restrictive pulmonary disease.
The most common extra-articular manifestation is acute, unilateral anterior uveitis. Diagnosis is primarily based on symptoms and x-ray of the sacroiliac joints, with HLA-B27 testing and MRI reserved for inconclusive cases. There is no curative treatment, but regular physiotherapy can slow progression of the disease. Additionally, NSAIDs and/or tumour necrosis factor-α inhibitors may improve symptoms. In severe cases, surgery may be considered to improve quality of life. The spine adopts a bamboo shape, not lordosis. The pain usually improves as the day progresses. leg raise test causes pain in cases of meningitis etc not in this case.

Pneumocystis carinii pneumonia, is an opportunistic fungal lung infection occurring almost exclusively in immunocompromised individuals. In 50% of cases, PCP is the first manifestation of AIDS (acquired immune deficiency syndrome), but it may be caused by other immunodeficiency disorders. PCP should be suspected in patients with a history of progressive dyspnoea and a dry cough with resistance to standard antibiotic treatment. Signs that support this diagnosis include a CD4 count < 200/μL, an increased beta-D-glucan level, and diffuse bilateral infiltrates on chest x-ray. Management of PCP includes high-dose trimethoprim/sulfamethoxazole (TMP/SMX), treatment of the underlying immunodeficiency disorder, and steroids in the case of severe respiratory insufficiency. TB is less likely to be present in this case as ESR is relatively low and chest x-ray appeared normal.

Hereditary C1 inhibitor deficiency leads to recurrent angioedema without urticaria or pruritus. Physical triggers include dental work, surgery or intubation. Medical triggers include angiotensin-converting enzyme (ACE) inhibitor, tamoxifen, oestrogen-containing medications (e.g., hormone replacement therapy and oral contraceptives). It is diagnosed on the basis of low levels of C1 esterase inhibitor or elevated levels of dysfunctional C1 esterase inhibitor. C4 levels are low between attacks. IgE levels, eosinophils, skin prick tests and RASTs are helpful in other allergic conditions and asthma but not of use in this case.

It is a metabolic bone disease caused by defective osteoclastic resorption of immature bone. Osteopetrosis is also known as marble bone disease. Osteoclasts are unable to adequately acidify bone matrix. Impaired bone resorption leads to overly dense bone that is more likely to fracture. It is usually treated with bone marrow transplant and high dose calcitriol.

Lateral epicondylitis (tennis elbow) is an overuse injury of the hand and finger extensor tendons that originate in the lateral humeral epicondyle that occurs following repeated or excessive pronation/supination and extension of the wrist (e.g., in racquet sports). Clinical features include pain and tenderness over the lateral epicondyle and along extensor muscles and thickening of the tendons. The examiner holds the patient’s hand with the thumb placed over the lateral epicondyle – The patient makes a fist, supinates the forearm, deviates radially, and extends the fist against the examiner’s resistance which will result in pain over the lateral epicondyle. Conservative treatment includes rest, physiotherapy and orthotic braces. If this fails corticosteroids and lidocaine injections are employed. Surgery is indicated in patients with persistent symptoms despite 6 months of conservative treatment. Excision of abnormal tendon tissue; longitudinal incisions (tenotomies) in scarred and fibrotic areas to promote healing.

Prevention of osteoporosis associated with chronic glucocorticoid therapy is done by administrating bisphosphonates. Oral bisphosphonates are indicated for patients aged above 65 who have been on steroid therapy for over 3 months, so as to reduce the risk of steroid induced osteoporosis. HRT is usually done in post menopausal women who have oestrogen related bone resorption.

Most patients with psoriatic arthritis present with monoarthritis or asymmetric oligoarthritis. The most common form of the disease is the one involving a few joints of the peripheral skeleton with a distinct asymmetry of symptoms. Involvement of the smaller joints of the hands and feet, especially distal interphalangeal joints, seems to be a characteristic feature. Arthritis mutilans is a rare and severe complication of psoriatic arthritis.

Reactive arthritis, (formerly known as Reiter’s syndrome), is an autoimmune condition that occurs after a bacterial infection of the gastrointestinal or urinary tract. It is categorized as a seronegative spondylarthritis because of its association with HLA-B27. Reactive arthritis primarily affects young men and usually presents with musculoskeletal or extra‑articular symptoms. The characteristic triad consists of arthritis, conjunctivitis, and urethritis. The diagnosis is based on clinical features such as patient history and physical examination; there are no specific tests for reactive arthritis. Treatment is primarily symptomatic and consists of the administration of NSAIDs, as most patients recover spontaneously. extraarticular dermatologic manifestations include skin lesions of the glans resembling psoriasis (balanitis circinata); hyperkeratinisation of the palms and soles (keratoderma blenorrhagicum), oral ulcers. There usually is no rash and the diagnosis is unrelated to the presence of rheumatoid factor. This is a clinical diagnosis.

Arthrocentesis should usually be done when there is a suspicion of pseudogout or septic arthritis as in this case which leads to an early diagnosis and prompt treatment. Polarized microscopy demonstrates weakly positively birefringent rhomboid crystals which are blue when parallel to light and yellow when perpendicular to light. Elevated serum uric acid levels that cause gout are usually found after large consumption of alcohol or meat, or post surgery. Autoimmune diseases like SLE, RA etc require an autoimmune screen.

Drug induced lupus typically presents with pulmonary involvement and no renal or neurological involvement. Hence glomerulonephritis would be highly unlikely in this case. Rash and arthralgias are classic presentations. Pleurisy can be present as pulmonary involvement may occur with DILE.

An overall concordance rate in monozygotic twins was documented to be 25% as compared to dizygotic twins with 3%. First degree relatives have a chance of around 3% of developing the disease. Caucasians show an increase frequency of HLA-B8. The Japanese lupus patients had a stronger association with HLA-DR2.

Angioedema secondary to C1 inhibitor deficiency has been rarely reported to be associated with systemic lupus erythematosus. A genetic defect of C1 inhibitor produces hereditary angioedema, which is usually presented with cutaneous painless oedema, but oedema of the genital area, gastrointestinal and laryngeal tracts have also been reported. In lupus patients, angioedema may be the result of an acquired type of C1 inhibitor deficiency, most probably due to antibody formation directed against the C1 inhibitor molecule.

The patient most likely has reactive arthritis which is usually diagnosed on history and clinical examination. The classic triad of symptoms include conjunctivitis, urethritis and arthritis. Arthritis and conjunctivitis may occur 4-6 weeks after a gastrointestinal or genitourinary infection. Arthritis usually occurs acutely, mostly involving the lower limb and is asymmetrical. Blood culture, urine culture and arthrocentesis (joint aspiration) will not yield positive results.

Blood pressure should be routinely measured as leflunomide may cause hypertension and thus an increase in BP. It doesn’t cause changes in blood sugar levels, peak expiratory flow rate or haematuria.

Diagnosis of RA is mainly based on clinical features (e.g., morning stiffness, symmetrical joint swelling) and laboratory tests (e.g., anti-CCP). Rheumatoid factor is not very specific for this disease and hence has low reliability. X-ray findings (e.g., soft tissue swelling or joint space narrowing) occur late in the disease and are therefore not typically used for diagnosis.

Although microscopic analysis of joint fluid aspirate and culture is the basis of septic arthritis diagnosis and should usually be collected before the administration of antibiotics, the patient is showing signs of septic shock. A delay in antibiotics might lead to worsening of symptoms so they are administered first.

A typical attack may last less than an hour but can also persist for longer. In primary Raynaud’s, attacks are more likely symmetric, episodic, and without evidence of peripheral vascular disease. Patients more commonly have a negative ANA and normal inflammatory markers. There should be no evidence of tissue gangrene, digital pitting, or tissue injury in primary Raynaud’s. In contrast, patients with secondary Raynaud’s will describe attacks that are more frequent, painful, often asymmetric and may lead to digital ulcerations.

Osteomalacia is a condition due to defective mineralization of osteoid. Occurs as a result of Vitamin D deficiency secondary to poor dietary intake and sun exposure, malabsorption e.g., inflammatory bowel disease and gastrointestinal bypass surgery. Radiological findings include reduced bone mineral density (a non specific finding), inability to radiologically distinguish vertebral body trabeculae (the film appears poor quality), looser pseudo fractures, fissures, or narrow radiolucent lines (these are the characteristic findings). Osteolytic or punched out lesions may be seen with multiple myeloma and bony metastases. Areas of sclerosis may be observed with conditions like osteosclerosis and Paget disease. A Brodie abscess is a subacute osteomyelitis, which may persist for years before progressing to a chronic, frank osteomyelitis.

Anti–Mi-2 antibodies are highly specific for dermatomyositis, but sensitivity is low; only 25% of patients with dermatomyositis demonstrate these antibodies. A positive antinuclear antibody (ANA) finding is common in patients with dermatomyositis, but is not necessary for diagnosis. Anti-Jo-1 antibodies are mostly associated with polymyositis. Anti Scl-70 antibodies and anti centromere antibodies are most commonly found in systemic scleroderma.

Reactive arthritis, (formerly known as Reiter’s syndrome), is an autoimmune condition that occurs after a bacterial infection of the gastrointestinal or urinary tract. It is categorized as a seronegative spondylarthritis because of its association with HLA-B27. Reactive arthritis primarily affects young men and usually presents with musculoskeletal or extra‑articular symptoms. The characteristic triad consists of arthritis, conjunctivitis, and urethritis. The diagnosis is based on clinical features such as patient history and physical examination; there are no specific tests for reactive arthritis. Treatment is primarily symptomatic and consists of the administration of NSAIDs, as most patients recover spontaneously. Dermatologic manifestations include skin lesions of the glans resembling psoriasis (balanitis circinata); hyperkeratinisation of the palms and soles (keratoderma blenorrhagicum)

Drug induced lupus is usually positive for antinuclear and antihistone antibodies, typically without renal or neurologic involvement. However, pulmonary involvement is common. Drugs that can induce lupus include isoniazid, hydralazine, procainamide, chlorpromazine, and other anticonvulsants.

People with Systemic Juvenile Idiopathic Arthritis (also known as Stills disease) can have recurrent fevers, a macular rash, joint pain, joint deformities, an enlarged liver and/or spleen, and can occasionally have polyserositis, lung involvement or pericardial effusions. Rheumatoid factor and antinuclear antibodies are usually negative. Treatment is with non-steroidal anti-inflammatory drugs (NSAIDs) and the prognosis is better than for adult rheumatoid arthritis.
In pauciarticular Still’s disease, antinuclear antibodies are present. Large joints are affected and most patients develop classic features of seronegative spondylarthritis.

DGI presents as two syndromes: 1) a bacteremic form that includes a triad of tenosynovitis, dermatitis, and polyarthralgias without purulent arthritis and 2) a septic arthritis form characterized as a purulent arthritis without associated skin lesions. Many patients will have overlapping features of both syndromes. Time from infection to clinical manifestations may range from 1 day to 3 months. There is no travel history and the rash of Lyme disease is not purulent. Reactive arthritis presents with conjunctivitis, urethritis and arthritis usually with a red hot tender and swollen joint.

Osteoblastic (or sclerotic) bony metastases, characterized by deposition of new bone, present in prostate cancer, carcinoid, small cell lung cancer, Hodgkin lymphoma or medulloblastoma. The other cancers listed in the options are osteolytic.

The most likely diagnosis is chronic tophaceous gout, which is classically associated with chronic renal impairment or long term diuretic therapy. There is tophus formation due to urate crystal deposition in and around the joint. These tophi can also form in the bones and soft tissues. Renal manifestations with uric acid include nephrolithiasis and uric acid nephropathy. Whenever there is an acute on chronic attack of gout, the inciting cause must be established and in case of diuretic use, they should be immediately replaced with another medication. Allopurinol is never started during an acute episode. it is first allowed to settle before administration of allopurinol. Although dietary restriction must be observed in people with a propensity of gout, this scenario clearly presents diuretics as the cause.

Temporal arteritis is a chronic large- and medium-sized vessel vasculitis that typically involves the temporal arteries. Classical symptoms include temporal headaches, jaw claudication, amaurosis fugax. Physical exam shows scalp tenderness, palpation of the temporal area may demonstrate an absent pulse, knot-like swelling and vision loss. Lab results reveal an increased erythematous sedimentation rate and C-reactive protein. Temporal artery biopsy confirms the diagnosis. Management approach: high-dose systemic corticosteroids should be promptly administered even before the diagnosis is established. Temporal artery biopsy confirms the diagnosis. Inability to manage this or administer glucocorticoids might lead to development of blindness.

Rheumatoid arthritis is both common and chronic, with significant consequences for multiple organ systems. Anti-cyclic citrullinated peptide (anti-CCP) antibody testing is particularly useful in the diagnosis of rheumatoid arthritis, with high specificity, presence early in the disease process, and ability to identify patients who are likely to have severe disease and irreversible damage. However, its sensitivity is low, and a negative result does not exclude disease. Anti-CCP antibodies have not been found at a significant frequency in other diseases to date, and are more specific than rheumatoid factor for detecting rheumatoid arthritis. The other factors that are mentioned do not play a key prognostic role.

Cubital Tunnel Syndrome is a condition that involves pressure or stretching of the ulnar nerve (also known as the “funny bone” nerve), which can cause numbness or tingling in the 4th and 5th digit, pain in the forearm, and/or weakness in the hand. Epicondylitis of the elbow is a condition associated with repetitive forearm and elbow activities. Both lateral epicondylitis (commonly known as tennis elbow) and medial epicondylitis (commonly known as golfer’s elbow) are characterized by elbow pain during or following elbow flexion and extension. Radial tunnel syndrome is a set of symptoms that include fatigue or a dull, aching pain at the top of the forearm with use. Although less common, symptoms can also occur at the back of the hand or wrist.

Scleroderma renal crisis (SRC) is a rare but severe complication in patients with systemic sclerosis (SSc). It is characterized by malignant hypertension, microangiopathic haemolytic anaemia with schistocytes and oligo/anuric acute renal failure. SRC occurs in 5% of patients with systemic scleroderma, particularly in the first years of disease evolution and in the diffuse form. Patients may develop symptoms of fluid overload.

Arthritis mutilans is a rare (occurs in only 5% of the patients) and extremely severe form psoriatic arthritis characterized by resorption of bones and the consequent collapse of soft tissue. When this affects the hands, it can cause a phenomenon sometimes referred to as ‘telescoping fingers.’ The associated nail changes are also characteristic of arthritis.

Rheumatoid arthritis is an inflammatory systemic disease associated with some extraarticular manifestations. Keratoconjunctivitis sicca, episcleritis, scleritis, corneal changes, and retinal vasculitis are the most common ocular complications among extraarticular manifestations of RA. The overall prevalence of keratoconjunctivitis sicca also known as dry eye syndrome among patients of RA is 21.2% and is the most common with sense of grittiness in the eyes.

If the INR in the range of 2-3 has still resulted in thrombosis, the target INR is increased to 3-4. However, because the risk of bleeding increases as the INR rises, the INR is closely monitored and adjustments are made as needed to maintain the INR within the target range.

Azathioprine therapy can cause acute myelosuppression. Toxicity is in part caused by the incorporation of azathioprine-derived 6-thioguanine nucleotides (6-TGN) into deoxyribonucleic acid (DNA). The enzyme thiopurine methyltransferase (TPMT) plays an important role in azathioprine catabolism.

Anti ds-DNA antibodies are very specific for SLE and their presence most often indicates systemic spread of the disease. These antibodies are present in about 30 percent of the total cases of SLE.

NSAIDs are considered as the first line of treatment for managing pain and stiffness associated with ankylosing spondylitis. Other useful medications include TNF-alpha inhibitors. Other drugs like paracetamol, colchicine, and steroids are not routinely used. Bilateral total hip replacement might be indicated in advanced disease contrary to complicated spinal surgery.

The patient most likely suffers from antiphospholipid syndrome. The clinical criteria consist of vascular thrombosis and pregnancy morbidity. Vascular thrombosis is defined as one or more clinical episodes of arterial, venous, or small-vessel thrombosis in any tissue or organ confirmed by findings from imaging studies, Doppler studies, or histopathology. ASD, VSDs would cause paradoxical emboli and stroke, however the recurrent pregnancy loss in this case is strongly suggestive of antiphospholipid syndrome. The ECG would be normal in most cases associated with anti phospholipid syndrome.

The patient has presented with signs of small cell lung cancer. The associated proximal muscle weakness is most probably due to dermatomyositis which occurs as a paraneoplastic syndrome associated with lung carcinoma. In most cases, the first symptom is a distinctive skin rash on the face, eyelids, chest, nail cuticle areas, knuckles, knees or elbows. The rash is patchy and usually a bluish-purple colour. Corticosteroids are helpful in the management of the cutaneous changes and muscle weakness.

Bilateral erosions of the sacroiliac joints on pelvic radiographs of patients with ankylosing spondylitis are an important feature of the modified New York classification criteria. Although HLA-B27 is commonly associated with AS, it can also be found in normal individuals. Back stiffness is worse in the morning and gets better as the day progresses. Tenderness and limited lumbar motion can be associated with other spine problems as well and is not characteristic of rheumatoid arthritis.

To confirm the diagnosis, especially in patients with negative anti-Ro or anti-La antibodies, labial gland biopsy is done. In performing a labial biopsy, the surgeon typically makes a shallow 1/2 inch wide incision on either side of the inner lip after numbing the area with a local anaesthetic. Schirmer’s test determines whether the eye produces enough tears to keep it moist. This test can be done for ocular symptoms of Sjogren syndrome but is of no diagnostic importance as it can be positive with many other diseases.

Old age, persistent backache, weight loss, and osteosclerotic lesions make prostatic adenocarcinoma the most likely diagnosis. The sequelae include severe pain, pathological fractures, hypercalcemia and cord compression. Prostatic adenocarcinoma is detected by elevated levels of prostate specific antigen. Positive serology for borrelia burgdorferi would hint at Lyme disease which does not cause osteosclerotic bone lesions, neither would Neisseria gonorrhoeae have such a presentation.

A high level of anti-dsDNA in the blood is strongly associated with lupus and is often significantly increased during or just prior to a flare-up. When the anti-dsDNA is positive and the person tested has other clinical signs and symptoms associated with lupus, it means that the person tested likely has lupus. This is especially true if an anti-Sm test is also positive.

In the evaluation of someone with lupus nephritis, a high level (titre) of anti-dsDNA is generally associated with ongoing inflammation and damage to the kidneys.

A very low level of anti-dsDNA is considered negative but does not exclude a diagnosis of lupus. Only about 65-85% of those with lupus will have anti-dsDNA.

Low to moderate levels of the autoantibody may be seen with other autoimmune disorders, such as Sjögren syndrome and mixed connective tissue disease (MCTD).

Rheumatoid factor is an IgM antibody that targets the Fc portion of IgG.