Mikulicz’s syndrome is the benign persistent swelling of lacrimal and parotid or submandibular glands due to lymphocytic infiltration. When no specific cause is found it is called Mikulicz’s disease; and if secondary to a disease like sarcoidosis or tuberculosis, it is termed as Mikulicz’s syndrome.

In this case, where it is likely secondary to sarcoidosis given the patient’s chest X-ray findings it is termed Mikulicz’s syndrome.

Mikulicz disease and syndrome have the same clinical picture and it comprises a triad of:

* Symmetrical enlargement of all salivary glands

* Narrowing of the palpebral fissures due to enlargement of the lacrimal glands.

* Dryness of the mouth.

This is a classic example of gout which most commonly affects the metatarsal-phalangeal joint of the big toe. Most attacks are sudden and can be managed with NSAIDs such as naproxen.

They are to be continued until 1 to 2 days after the attack has been resolved. Both NSAIDs and colchicine are considered first-line management in acute gout attacks which makes this question very difficult to answer.

NSAIDs are generally more often prescribed in primary practice for gout and are more tolerable since colchicine is known for its side effects which include diarrhoea and nausea.

So in the exam, if you were given the choice between the two and the patient is a young adult with no history of gastritis or asthma, pick NSAIDs. Always keep in mind that NSAIDs may exacerbate asthma and may worsen gastritis.

They should also not be used in patients with renal insufficiency. Proton pump inhibitors are often prescribed together with NSAIDs for gastric protection, especially in the elderly where use of NSAIDs should be with caution.

Allopurinol is used to prevent recurrence of gout and it is not used to treat ACUTE gout.

Corticosteroids do have a role in gout that does not respond to NSAIDs or colchicine. They can be given as a 5-day course of oral tablets, injected into the joint, or injected intramuscularly.

Drinking too much alcohol can cause uric acid to build up and cause gout.

The two top differentials here that one should think about are polymyositis and polymyalgia rheumatica. Proximal muscle weakness and raised creatinine kinase point towards polymyositis.

ESR is elevated in 50% of cases of polymyositis but correlates poorly with disease activity and response to therapy.

In this stem, ESR is not elevated enough to consider polymyalgia rheumatica. Also, in polymyalgia rheumatica, and creatinine kinase would not be raised. One of the criteria for the diagnosis of polymyalgia rheumatica is having an ESR >30 mm/h or CRP >6 mg/mL which we do not see in this stem.

Hence, the best answer here is polymyositis.

Polymyositis :

- First presents with difficulty with tasks that involve the proximal muscles: lifting objects, combing hair, and getting up from the chair.

- The weakness is usually symmetric and diffuse, involving the proximal muscles of the neck, shoulders, trunk, hips, and thighs, the lower limb muscles tending to be clinically symptomatic first.

- Fatigue, myalgia, and muscle cramps may also be present.

Investigation:

• Elevation of creatine kinase.

• Aldolase levels may also be raised.

• Autoantibodies: Anti-Jo-1 antibodies.

Note that these are more common in patients with polymyositis than in patients with dermatomyositis.

• Muscle biopsy can be diagnostic.

Treatment - Involves steroids.

Temporal arteritis (TA) is the suspected diagnosis here. The erythrocyte sedimentation test (ESR) is the first test to be done in patients suspected to have TA. Since the question asks for the most appropriate “initial” investigation, the answer would be ESR.

If the question had asked for the most appropriate “definitive” investigation, the temporal artery biopsy would be the answer.

Temporal Arteritis, also known as giant cell arteritis, is a vasculitis affecting the large arteries that supply the head, eyes, and optic nerves.

New-onset headache in any patient older than 50 years prompts consideration of this diagnosis, which if left untreated may result in permanent vision loss. The most common symptoms of giant cell arteritis are headache and pain that usually occurs in one or both temples.

Other common symptoms include scalp tenderness, pain when combing hair, jaw claudication (jaw pain when chewing), decreased vision or blurry vision, tongue numbness, or, rarely, sudden loss of vision.

Sometimes the patient may have proximal stiffness (neck, arms, hips) due to polymyalgia rheumatica, an coexisting condition with TA.

The erythrocyte sedimentation test (ESR) is the first test to be done in patients suspected to have TA.

The diagnosis is always confirmed by a biopsy of the temporal arteries in which the characteristic giant cells are demonstrated. In the patient whom you suspect to have, if the ESR is elevated, corticosteroids should be started immediately, before the temporal artery biopsy is performed.

Occasionally they may ask which medication can be added to corticosteroids for treatment of GCA.

Add on low-dose aspirin. Aspirin 75 mg daily has been shown to decrease the rate of visual loss and strokes in patients with GCA.

Severe unilateral headache with scalp tenderness and the absence of any neurological symptoms suggest that the lady is suffering from Temporal Arteritis or Giant Cell Arteritis.

It is an inflammatory condition and is treated with corticosteroids. This is a high-yield question in exam and may be asked for either the diagnosis, pathophysiology, or treatment.

De Quervain’s tenosynovitis, also known as “Washerwoman’s Sprain, Mummy Thumb, and Gamer’s Thumb’, is caused by the inflammation of the extensor pollicis brevis and the abductor pollicis longus due to repetitive stress injury.

It commonly occurs in women following pregnancy due to how the baby is lifted and held.

The presentation is in line with septic arthritis where rheumatoid arthritis is a risk factor. The most important diagnostic test in patients presenting with acute arthritis is an examination of the synovial fluid which was performed in this stem.

Empirical treatment should be started in septic arthritis before the results of the culture are obtained.

Intravenous flucloxacillin 1 to 2 g every 6 hours is the most common antibiotic to start with.

SEPTIC ARTHRITIS :

The two most common organisms that cause septic arthritis are:

- Staphylococcus aureus. 

- The most common pathogen for septic arthritis overall : Neisseria gonorrhoeae. 

Seen in young, sexually active adults.

Most cases of septic arthritis are due to haematogenous spread during transient bacteremia but can also be introduced by a skin lesion that penetrates the joint or by local spread from a contiguous infected site.

Risk factors :

- Prior joint damage (rheumatoid arthritis, gout, osteoarthritis) It is particularly important to remember rheumatoid arthritis as a risk factor as it is commonly asked

- Immunodeficiency states (HIV, corticosteroid use)

- Diabetes.

Presentation :

- Single swollen, red joint with pain on active or passive movement

- Fevers and rigors

*** Remember this triad of fever, pain, and impaired range of motion

Investigations :

- Aspiration of synovial fluid : Sent for gram staining, leukocyte count, microscopy, and culture-blood cultures.

 *** Remember most cases of septic arthritis are due to hematogenous spread

Management :

- Flucloxacillin for 4 to 6 weeks

- If penicillin-allergic use clindamycin

- If gonococcal arthritis : Use cefotaxime or ceftriaxone

- If the infection not responding to antibiotics. Perform repeated percutaneous aspiration.

In general, intravenous antibiotics are used for 7 days until the swelling subsides and blood cultures become negative. This is followed by a 4-week course of oral antibiotics.

Sjogren's syndrome is an autoimmune disorder affecting exocrine glands resulting in dry mucosal surfaces.

Sjogren syndrome may be seen alone (primary) or in association with other autoimmune diseases (secondary) such as rheumatoid arthritis, primary biliary cirrhosis, or systemic lupus erythematosus.

As Sjogren's syndrome progresses, it becomes a systemic disease involving major organs (lungs, kidneys, etc.) and may eventually evolve into a lymphoproliferative disease - malignant Lymphoma.

Note that Sjogren's syndrome is much more common in females (ratio 9:1)

Features :

Dry eyes: keratoconjunctivitis sicca

- Patients complain of itchy eyes, and a sandy feeling under their eyes (because of decreased lacrimal production) & dry mouth.

- Patients may complain of difficulty swallowing food, vaginal dryness.

- Bilateral enlargement of the parotid glands

Investigation :

- Schirmer's test will show decreased tear production

- Rose Bengal stain will document corneal ulcerations

- ANAs will be positive and specifically anti-Ro (SSA) and anti-La (SSB)

- Rheumatoid factor (RF) positive in nearly 100% of patients.

Management : 

There is no cure for Sjogren's syndrome. Artificial tears may help with dry eyes.

Please see Q-9

Low dose aspirin is increasingly being recommended for people with a history of giantcell arteritis. It has been found to decrease the rate of visual loss and strokes in patients with GCA be started immediately, before the temporal artery biopsy is performed.

Reactive arthritis is caused when a joint reacts to an infection elsewhere in the body. The infection that triggers reactive arthritis is not actually in the joint itself. Most often, these bacteria are in the genitals (Chlamydia trachomatis) or the bowel (Campylobacter, Salmonella, Shigella, and Yersinia).

Unlike septic arthritis, fever is not a typical feature of reactive arthritis as it is not caused by an active infection although it is seen in some cases.

Reactive arthritis :

It is a form of seronegative spondyloarthritis clinically associated with back pain, migratory oligoarthritis, and extra-articular symptoms that typically follow gastrointestinal or urogenital infection.

Presentation :

- Develops 2-4 weeks after an initial infection which may have been sexually acquired or gastrointestinal in origin

- An asymmetrical, predominantly lower extremity, oligoarthritis is the major presenting symptom (usually knees and ankles)

- Skin (circinate balanitis, keratoderma blennorrhagicum, erythema nodosum)

- The complete Reiter's triad of urethritis, conjunctivitis, and arthritis may occur.

Mnemonic: "Can't see, can't pee, can't climb a tree."

- Can't see – Conjunctivitis - Can't pee – Urethritis - Can't climb a tree – Arthritis.

Bendroflumethiazide is a thiazide diuretic that is well known to cause symptomatic hyperuricemia which can precipitate an acute attack of gout.

Uric acid levels can be falsely low during an attack and SHOULD NOT be measured until 4 weeks after an acute attack.

In summary, you may sometimes see a raised serum uric acid level in acute gout, however, not all people suffering from gout demonstrate raised serum uric acid levels and this is not an accurate test to diagnose the condition.

The gold standard is still an aspiration of synovial fluid and the detection of crystals on microscopy. Although aspiration of synovial fluid is the gold standard, it is rarely done. They can be difficult and painful and one needs to remember that the majority of gout are diagnosed in GP clinics where time is of the essence.

All the tests above may be ordered when investigating for SLE however antinuclear antibody (ANA) is the most sensitive of all the tests in the options given and is usually ordered alongside FBC, U&E, LFTs, ESR, and CRP when suspecting SLE.

Anti-dsDNA may be more specific but it is less sensitive compared to ANA. As this question is asking for an initial investigation, ANA would be more appropriate as it is more sensitive.

Churg-Strauss Syndrome :

A rare diffuse vasculitic disease affecting coronary, pulmonary, cerebral, abdominal visceral, and skin circulations. The vasculitis affects small- and medium-sized arteries and veins and is associated with asthma.

The cardinal manifestations of Churg-Strauss syndrome are asthma, eosinophilia, and lung involvement.

Investigations :

- ANCA; 30-40% of patients are p-ANCA positive (antimyeloperoxidase antibodies)

- Eosinophilia and anaemia on the FBC

- Elevated ESR and CRP

- Elevated serum creatinine

- Increased serum IgE levels

- CXR: pulmonary opacities, transient pulmonary infiltrates, and bilateralmultifocal consolidation

- High-resolution CT: Ground-glass attenuation

- Biopsy: small necrotising granulomas, as well as necrotising vasculitis (found especially in the lung).

The diagnosis here is acute gout which is precipitated by bendroflumethiazide.

The diagnosis is quite clear here.

Systemic sclerosis may present with pain in the hands when exposed to cold (Raynaud's phenomena), shortness of breath (pulmonary fibrosis), and dysphagia (oesophageal dysmotility). Systemic sclerosis Is one of the causes of dysphagia.

Features of CREST syndrome namely :

Calcinosis

Raynaud's phenomenon

Esophageal dysmotility

Sclerodactyly

Telangiectasia

As well as oesophageal dysmotility the lower oesophageal sphincter (LES) pressure is decreased. This contrasts with achalasia where the LES pressure is increased.

Prednisolone should be started when you suspect temporal arteritis if ESR is elevated. Do not wait till you get a temporal artery biopsy.

This is a multi-step question.

Let's break it down:

- This man has had a myocardial infarction

- He is now suffering from heart failure

- He is also suffering from a red, swollen knee joint.

By mentioning that this man has heart failure, they are alluding to the fact that he was probably put on a diuretic during his stay in the hospital.

Loop diuretics (such as Furosemide) are used first-line for cardiac failure. A thiazide diuretic may be added to a loop diuretic in the case of severe cardiac failure. We do not know what type of diuretic this man has been put on but he was probably put on a thiazide diuretic.

To diagnose gout, we MUST do a joint aspirate. Since there was no mention of this man having a fever, there is no need to do a joint aspirate culture. Joint microscopy would be ideal since we would be able to visualize the MSU crystals of gout.

You may sometimes see a raised serum uric acid level in acute gout, however, not all people suffering from gout demonstrate raised serum uric acid levels and this is not an accurate test to diagnose the condition.

Keep in mind that when the stem says “the single best method for confirming the diagnosis”, it is always a biopsy.

The diagnosis here is Churg-Strauss syndrome. Churg-Strauss syndrome is now commonly known as eosinophilic granulomatosis with polyangiitis or allergic granulomatosis. 30-40% of patients are perinuclear staining (p-ANCA) positive.

Please see Q-9

The elderly lady with giant cell arteritis is getting high-dose steroids which can lead to demineralization and osteopenia or osteoporosis. So to prevent this bisphosphonates are given. The diagnosis here is clear. She has temporal arteritis.

This is not a clear-cut question and the most likely diagnosis here is limited systemic sclerosis. Both limited and diffuse systemic sclerosis may present with pain in the hands when exposed to cold (Raynaud's phenomena).

The classical features of Raynaud's phenomenon are episodic pallor of the digits due to ischaemia, followed by cyanosis due to deoxygenation, and then followed by redness with pain and tingling which is when there is reperfusion.

The presence of the pink and red dots on the lips and tips of fingers represent telangiectasia which is seen as part of the CREST syndrome in limited systemic sclerosis.

Arthralgia is usually seen more in diffuse systemic sclerosis which goes against the answer of limited systemic sclerosis.

In limited systemic sclerosis, the complaint is usually the occasional joint stiffness rather than arthralgia. However, given the slow progression of the disease and no other features of organ involvement, limited systemic sclerosis is likely the answer.

Rheumatoid arthritis is also a potential differential because it is another cause of Raynaud's phenomenon and would explain symmetrical peripheral arthropathy. Nonetheless, rheumatoid arthritis is a rare cause of Raynaud’s phenomenon and an even rarer cause of telangiectasia. Given the stem, the best answer remains as limited systemic sclerosis.

X-rays of the sacroiliac joints are the most useful investigation. It would show evidence of sacroiliitis which is the earliest finding. 

ESR levels correlate with disease activity and are a good blood test to order when thinking about ankylosing spondylitis but an X-ray is still a better test to perform to diagnose ankylosing spondylitis.

ANKYLOSING SPONDYLITIS :

An HLA-B27-associated spondyloarthropathy that primarily involves the axial skeleton (ie sacroiliitis and spondylitis)

Features :

- Young man (usually before age 30) presenting with lower back pain and stiffness

- Stiffness which is worse in the morning and improves with exercise

- A strong association with HLA-B27 exists

- There is often tenderness of the sacroiliac joints or a limited range of spinal motion. Examination : Schober's test. 

- Another important feature is anterior uveitis. Presents with an acutely painful red eye and severe photophobia

- Aortic regurgitation.

Investigations :

- Plain X-ray of the sacroiliac joints. This is the most useful investigation. It would show evidence of sacroiliitis which is the earliest finding. Later findings there significant chronic spine inflammation including a “bamboo spine” and squaring of the vertebral bodies.

- Do not use HLA-B27 to make the diagnosis as it is also positive in 10% of normal patients.

Management:

First line - NSAIDS.

Second line - Anti-TNF therapy.

Middle aged man with asthma and skin nodules, one has to consider Churg-Strauss syndrome despite the rarity.

The diagnosis here is Churg-Strauss syndrome. The incidence of Churg-Strauss syndrome is rare thus it is also rarely asked in exam. However, one might see this topic now and then.

Seronegative spondyloarthropathy is a group of diseases involving the axial skeleton.

The term “seronegative” refers to the fact that these diseases are negative for rheumatoid factor. This indicates that there is a different pathophysiological mechanism of disease when compared with rheumatoid arthritis.

Ankylosing spondylitis is one of the seronegative spondyloarthropathies and it is seen in this stem. The lower back pain and stiffness are consistent with ankylosing spondylitis.

He is around the correct age to develop ankylosing spondylitis as well. Ankylosing spondylitis usually occurs before the age of 30 years.

The eye manifestations are consistent with acute anterior uveitis which occurs in patients with ankylosing spondylitis. 

The chronic diarrhea, aphthous ulcers, and perianal skin tags are consistent with Crohn's disease. You need to remember that there is an increased incidence of ankylosing spondylitis in patients with inflammatory bowel disease (IBD). 

The blood results are also consistent with ankylosing spondylitis - Normochromic normocytic anemia of chronic disease - Elevated ESR and CRP levels correlating to disease activity.

Staphylococcus aureus is the most common bacteria infecting adult joints.

Remember, not all patients will have that classic butterfly rash over the nasal bridge and malar bones. Only half of them are usually present that way.

Morning stiffness and polyarticular, symmetrical arthralgia are often seen in patients with systemic lupus erythematosus. Fatigue and tiredness are common features too.

In this stem, the patient also presents with glomerulonephritis. ANCAs are associated with small vessel vasculitides including Wegener’s granulomatosis and sometimes Churg-Strauss syndrome.

The absence of ANCA cannot indicate the absence of disease but it gives us a clue of which are the less likely options in this question.

Ibuprofen is the best answer given the options.

Methotrexate and hydroxychloroquine are both DMARDs that help prevent disease progression in the long term but do not help with the immediate pain. Paracetamol is a good pain relief but it is not suitable for severe pain.

There are no contraindications to using ibuprofen and low-dose aspirin.

The diagnosis here is gout. NSAIDs are given for acute attacks.

This man was likely prescribed furosemide to combat the fluid overload from heart failure. Furosemide may raise uric acid levels and precipitate gout.

Gout most commonly affects the metatarsophalangeal joint of the first toe but question writers will not make questions that obvious and often they may describe it as left foot swelling and pain.

Other common precipitants of gout asked are :

• Excessive amounts of alcohol

• Thiazide diuretics 

Raynaud’s phenomenon is defined as episodes of pallor or cyanosis in response to cold or emotional stimuli.

Fingers or toes ache and change colour: pale (ischaemia), blue (deoxygenation), red (reactive hyperaemia).

The pallor is caused by vasoconstriction of blood vessels (arteries and arterioles) that results in reduced blood flow, while cyanosis is created by deoxygenation of slow-flowing blood. After rewarming the hands, the blood flow will rebound (hyperemia) and the skin will appear reddened or blushed.

It is common for patients with Raynaud’s phenomenon to complain of cold sensitivity and to have other areas of the skin involved, including the ears, nose, and lower extremities.

Episodes come as sudden attacks and are most often triggered by rapid changes in ambient temperature.

Primary Raynaud’s phenomenon (Raynaud’s disease) denotes a patient without an associated underlying disease.

Secondary Raynaud’s phenomenon is used to describe patients with a defined secondary or associated disease (scleroderma).

The presentation is in line with septic arthritis where rheumatoid arthritis is a risk factor. The most important diagnostic test in patients presenting with acute arthritis is an examination of the synovial fluid.

This patient has granulomatosis with polyangiitis (GPA), previously known as Wegener’s granulomatosis.

GPA is an idiopathic small to medium vessel vasculitis which appears around middle age. GPA can present with a host of signs and symptoms but in the exam, look for anything affecting the upper respiratory tract, the lungs, and the kidneys.

In a long stem such as this, you need to be able to pick out keywords.

* Upper respiratory tract

• Nosebleeds

• Crust around nostrils

• Chronic sinusitis 

• Haemoptysis

• Cough 

• Haematuria

Try to imagine a person bleeding from his nose, lungs, and kidneys.

Remember that cANCA usually test positive in GPA whereas pANCA is usually seen in Churg-Strauss syndrome. 

Chronic fatigue syndrome is a clinical syndrome involving severe fatigue, unrelated to exertion or triggered by minimal activity, and unrelieved by rest.

Features :

• Persistent or recurrent fatigue

• Fatigue unexplained by other conditions

• Difficulty sleeping

• Muscle and joint pain at multi-sites without evidence of inflammation

• Painful lymph nodes without pathological enlargement

• Headaches

• Cognitive dysfunction such as difficulty thinking, concentrating, or finding words.

Sometimes, patients may link their symptom onset to the start of a viral infection.

They remember the precise beginning as it appeared together with viral infection with a sore throat, fever, and tender lymph nodes but had continued for months. Before labeling the patient with chronic fatigue syndrome, be sure to investigate other autoimmune rheumatic diseases that cause pain and fatigue. 

Systemic lupus erythematosus (SLE) is an autoimmune disease.

In this disease, the immune system of the body mistakenly attacks healthy tissue. It can affect the skin, joints, kidneys, brain, and other organs.

Photosensitive butterfly erythematous rash on the face (malar rash) and arthropathy are the most common features. Here puffiness of face is due to lupus nephritis.

The classic ocular lesion in SLE is a white fluffy appearing lesion within the retina known as a cotton wool spot. The most common ocular manifestation in SLE is Keratoconjunctivits sicca.

Headache in an elderly lady and jaw claudication are suggestive of temporal arteritis.

Temporal arteritis is a condition in which the temporal arteries, which supply blood to the head and brain, become inflamed or damaged. It is also known as giant cell arteritis.

Blood ESR is very high in temporal arteritis and it is routinely done in the elderly with suggestive clinical features.

Henoch-Schönlein purpura is an acute immunoglobulin A (IgA)–a mediated disorder characterized by a generalized vasculitis involving the small vessels of the skin, the gastrointestinal (GI) tract, the kidneys, the joints, and, rarely, the lungs and the central nervous system (CNS).

It is the most frequent vasculitis in childhood. In HSP rash typically found in the buttocks, legs, and feet and may also appear on the arms, face, and trunk. HSP usually follows a sorethroat.

Systemic sclerosis.

Raynouds phenomena, pulmonary involvement, and oesophageal dysmotility are suggestive of systemic sclerosis. Systemic sclerosis, also called diffuse scleroderma is an autoimmune disease of the connective tissue. It is characterized by thickening of the skin caused by accumulation of collagen, and by injuries to small arteries.

Apart from skin involvement following organs are mostly affected in systemic sclerosis: the kidneys, heart, lungs, and gastrointestinal tract.

Diagnosis is by clinical suspicion, presence of autoantibodies (specifically anti-centromere and anti-scl70 / anti-topoisomerase antibodies) and occasionally by biopsy.

Elderly (age 62), right-sided headache and loss of vision are suggestive of temporal arteritis where elevated ESR is highly suggestive of this diagnosis.

Low-dose aspirin is increasingly being recommended for people with a history of giant cell arteritis. It has been found to be effective in preventing complications of giant cell arteritis, such as heart attacks or stroke.

Parotid swelling, difficulty talking and speaking (due to dryness or less saliva), eyes feeling gritty on waking in the morning due to dryness of eyes are suggestive of Sjogren's syndrome.

The elderly lady with giant cell arteritis is getting high-dose steroids which can lead to demineralization and osteopenia or osteoporosis. So to prevent this bisphosphonates are given.

Likely diagnosis is SLE with lupus nephritis for which autoantibody (anti ds DNA antibody) should be done.

Anti-histone antibody is present in 95% of cases of drug-induced lupus.

The most common drugs that cause drug-induced SLE are hydralazine, procainamide, quinidine, isoniazid, and minocycline.

Anti DNA antibodies to diagnose SLE.

Methotrexate is used as DMARDs (disease modifying anti rheumatic drugs) in rheumatoid arthritis to slow the disease progression which leads to folate deficiency anaemia.

Proximal muscle weakness (inability to climb the stairs) and raised CPK points towards the diagnosis of polymyositis.

Young man with early morning back pain and stiffness is suggestive of seronegative arthritis likely ankylosing spondylitis where iritis is a common association.

Diagnosis is rheumatoid arthritis. The ocular manifestation of rheumatoid arthritis is scleritis (red, painful eye).

X-ray sacroiliac joint shows fusion of both SI joints and thin, symmetrical syndesmophytes bridging the intervertebral disc spaces.

Back pain with early morning stiffness and uveitis points towards the diagnosis of ankylosing spondylitis.

Proxymal myopathy (not able to climb stairs), mild breathlessness (involvement of thoracic muscles or interstitial lung disease), dysphagia due to involvement of the oropharyngeal striated muscles and upper oesophagus) and raised CK suggests the diagnosis of Polymyositis.

An elderly lady with severe pain in the left half of her skull and pain around her jaw is suspicious of Giant cell or Temporal arteritis.

ESR if markedly raised supports the diagnosis of temporal arteritis.

 Limited systemic sclerosis.

Raynaud’s phenomena, telangiectasia, sclerodactyly indicates the diagnosis of CREST syndrome or Limited systemic sclerosis.

Mikulicz’s disease is a well-known disorder characterized by enlarged lacrimal and parotid glands caused by infiltration with lymphocytes. When no specific cause is found it is called Mikulicz’s disease; and if secondary to disease like sarcoidosis is termed as Mikulicz’s syndrome.

Features are suggestive of SLE. So most appropriate investigation from the given options is A. Anti ds DNA antibodies.

Psoriatic arthritis initially can affect the toes very similar to gout. There can be sacroiliitis and onycholysis. 

Henoch-Schönlein purpura (HSP) is an acute immunoglobulin A (IgA)–a mediated disorder characterized by a generalized vasculitis involving the small vessels of the skin, the gastrointestinal (GI) tract, the kidneys, the joints, and, rarely, the lungs and the central nervous system (CNS).

Rash on the trunk, abdominal pain, arthritis, proteinuria, and hematuria are well-known features of HSP.

Bisphosphonates.

Oral steroid can lead to decrease in bone mineral density. To overcome this bisphosphonates can be administered.

ARF and recurrent epistaxis and hemoptysis suggest Wegener’s granulomatosis.

Wegener's granulomatosis is a rare disease of uncertain cause that can affect people of all ages. It is characterized by inflammation in various tissues, including blood vessels (vasculitis), but primarily parts of the respiratory tract and the kidneys for which C ANCA is most specific.

Takayasu arteritis is a granulomatous inflammation of the aorta and its major branches.

Criteria: Age of 40 years or younger at disease onset. Claudication of the extremities. Decreased pulsation of 1 or both brachial arteries.

 Difference of at least 10 mm Hg in systolic blood pressure between arms. Bruit over 1 or both subclavian arteries or the abdominal aorta. Arteriographic narrowing or occlusion of the entire aorta, its primary branches, or large arteries in the upper or lower extremities that are not due to arteriosclerosis, fibromuscular dysplasia, or other causes.

Anticentromere would be present in limited scleroderma and Anti-Scl 70 would be present in diffuse scleroderma. Anti-DSDNA would be positive in SLE, along with Anti- Ro.

Anti-Ro is also positive in Sjogren’s syndrome and scleroderma.

This is a case of Polymyositis because none of the others would have the muscular weakness that is present in this case. The antibody of choice in polymyositis is Anti Jo1 antibody.

Anti centromere.

Dysphagia, Raynaud’s phenomenon, telangiectasia, painful lesions on fingertips are suggestive features of CREST syndrome or limited scleroderma. Anti-centromere antibodies are found in approximately 50-90% of patients with limited forms of scleroderma;

Anticentromere antibodies are present in 82-96% of patients with the CREST variant. The specificity of this test is 95%.

The symptoms described are characteristic of ankylosing spondylitis (lower back pain and stiffness which gets better after moving around and taking NSAIDS) the extra articular manifestations of AS is iritis.

This is a sample question and the given key is B. Reactive arthritis.

Sclerodactyly.

Patient is suffering from crest syndrome, sclerodactyly its one of the features.

PAN (polyarteritis nodosa) may affect nearly every organ system and thus it can present with a broad array of signs and symptoms.

These manifestations result from ischemic damage to affected organs, often the skin, heart, kidneys, and nervous system.

Constitutional symptoms are seen in up to 90% of affected individuals and include fever, fatigue, weakness, loss of appetite, and unintentional weight loss. Muscle and joint aches are common.

The skin may show rashes, swelling, necrotic ulcers, and subcutaneous nodules (lumps).

Skin manifestations of PAN include palpable purpura and livedo reticularis in some individuals. Abdominal pain may also be seen.