Rheumatology and Immunology Practice Questions

Q: A 25-year-old female develops serous otitis media of the left ear, accompanied by cough, occasional hemoptysis, hematuria, and epistaxis, over a period of one and a half months. Her hemoglobin is 7 g, blood pressure is >170/100 mmHg, proteinuria is ++++, and she is negative for rheumatoid factor and antineutrophil cytoplasmic antibody. What is the likely cause?

Goodpasture's syndrome. Goodpasture's syndrome - The presentation of recurrent hemoptysis (due to pulmonary hemorrhage), hematuria (due to glomerulonephritis), and a high blood pressure with severe proteinuria is highly consistent with Goodpasture's syndrome [4]. - Goodpasture's syndrome is an autoimmune disease characterized by antibodies against the glomerular basement membrane [2], affecting both the lungs and kidneys, often sparing other systems [4]. Wegener's granulomatosis - While it can cause hematuria, hemoptysis, and ear symptoms, Wegener's granulomatosis (now called Granulomatosis with Polyangiitis) is usually associated with a positive c-ANCA (a type of antineutrophil cytoplasmic antibody), which is negative in this patient [3]. - It typically involves necrotizing granulomatous inflammation of the upper and lower respiratory tracts, kidneys, and other organs, but the negative ANCA makes it less likely [4]. Rheumatoid arthritis - Rheumatoid arthritis primarily affects the joints and can have systemic manifestations, but it typically does not cause the severe presentation of hemoptysis, hematuria, and renal failure described. - The patient is also negative for rheumatoid factor, making rheumatoid arthritis highly unlikely. Rapidly proliferative glomerulonephritis - While rapidly proliferative glomerulonephritis (RPGN) can cause hematuria, proteinuria, and renal impairment, it is a broad category of diseases [1]. - The specific combination of prominent pulmonary hemorrhage (hemoptysis) and renal involvement (nephritic syndrome signs) points more specifically to Goodpasture's syndrome as the underlying cause of RPGN, given the clinical context [4].

Q: Anti-Ro antibodies are present in all except:

Mixed connective tissue disorder. **Mixed connective tissue disorder** - Anti-Ro antibodies are **not typically associated** with mixed connective tissue disorder (MCTD) [2]; **anti-U1-RNP antibodies** are the hallmark of MCTD [1]. - While patients with MCTD may occasionally have other autoantibodies, anti-Ro is not considered a diagnostic or distinguishing feature. *SLE* - Anti-Ro antibodies (also known as **SSA antibodies**) are present in approximately 25-50% of patients with **systemic lupus erythematosus (SLE)** [1]. - They are particularly associated with specific phenotypes of SLE, including **subacute cutaneous lupus erythematosus** and **neonatal lupus**. *Sjögren syndrome* - Anti-Ro antibodies are a **key serological marker** for Sjögren syndrome, found in 70-90% of patients [1]. - These antibodies are strongly associated with the characteristic **sicca symptoms** (dry eyes, dry mouth) and extraglandular manifestations of Sjögren syndrome. *Neonatal lupus* - Anti-Ro antibodies present in the mother can **cross the placenta** and cause **neonatal lupus** in the fetus. - This condition can lead to manifestations like **congenital heart block** and a transient rash in the newborn.

Q: A 20-year-old girl presents with abdominal pain and a purpuric rash all over the body, and the most probable diagnosis is?

Henoch-Schönlein Purpura (HSP). ***Henoch-Schönlein Purpura (HSP)*** - The classic triad of **abdominal pain**, **arthralgia** (often knee and ankle), and a **palpable purpuric rash**, particularly on the lower extremities and buttocks, strongly points to HSP [2]. - This is a **small vessel vasculitis** mediated by IgA immune complex deposition [1], [2]. *Hemolytic Uremic Syndrome (HUS)* - HUS is characterized by a triad of **microangiopathic hemolytic anemia**, **thrombocytopenia**, and **acute kidney injury**, often preceded by bloody diarrhea due to Shiga-toxin producing *E. coli* [3]. - While it can cause abdominal pain and purpura-like lesions (due to thrombocytopenia), the specific presentation here is more indicative of HSP. *Kawasaki Syndrome* - Kawasaki disease is a **medium-vessel vasculitis** primarily affecting young children, characterized by fever, conjunctivitis, oral mucosal changes, rash, lymphadenopathy, and extremity changes [4]. - It does not typically present with the same type of palpable purpuric rash or severe abdominal pain described in this case. *Immune Thrombocytopenic Purpura (ITP)* - ITP is an **isolated thrombocytopenia** (low platelet count) leading to increased bleeding tendency, presenting as petechiae, purpura, and mucosal bleeding. - While purpura is present, the additional finding of significant **abdominal pain** points away from isolated ITP and towards a systemic vasculitis like HSP.

Q: All are true regarding lupus anticoagulant except?

Thrombocytosis. ***Thrombocytosis*** - **Lupus anticoagulant** is associated with an **increased risk of thrombosis**, but it does not directly cause thrombocytosis (an elevated platelet count). - Patients with **antiphospholipid syndrome**, which involves lupus anticoagulant, can have normal or even low platelet counts (**thrombocytopenia**). [1] *Increased antibodies* - **Lupus anticoagulant** is an **autoantibody** (specifically, an antiphospholipid antibody) that interferes with coagulation tests in vitro, but it is associated with an increased risk of thrombosis in vivo. - The presence of this antibody is a key diagnostic criterion for **antiphospholipid syndrome**. *Rashes* - **Lupus anticoagulant** is often found in patients with **systemic lupus erythematosus (SLE)**, a condition known for various skin manifestations, including characteristic rashes (e.g., **malar rash**, discoid rash). [1] - While not directly caused by lupus anticoagulant, it is highly associated given the overlap between **SLE and antiphospholipid syndrome**. *Arterial thrombosis* - **Lupus anticoagulant** is a significant risk factor for both **arterial and venous thrombosis**, leading to complications like strokes, myocardial infarctions, and peripheral arterial occlusions. - It contributes to a **prothrombotic state** by activating endothelial cells and interfering with natural anticoagulant pathways.

Q: Lady presents with joint pain in both knees and low-grade fever, which occurs intermittently. On examination, she has a rash on sun-exposed areas. What is the clinical diagnosis?

Systemic lupus erythematosus (SLE). ***Systemic lupus erythematosus (SLE)*** - The combination of **polyarthralgia (joint pain)**, **low-grade fever**, and a **photosensitive rash** is highly characteristic of SLE. [1] - SLE is a **multisystem autoimmune disease** that can affect joints, skin, and cause constitutional symptoms like fever. [1] *Rheumatoid arthritis (RA)* - While RA causes **joint pain**, it typically presents with **symmetrical polyarthritis** primarily affecting small joints, and a photosensitive rash is not a common feature. [2] - **Fever** can be present in severe RA, but the triad of symptoms in this case points away from RA as the primary diagnosis. *Photodermatitis* - **Photodermatitis** is a skin inflammation caused by sunlight exposure, resulting in a rash. - This diagnosis does not account for the **joint pain** or **low-grade fever**, which are systemic manifestations. *Porphyria cutanea tarda* - This condition primarily affects the skin, causing **photosensitivity**, blistering lesions, and increased fragility, especially on sun-exposed areas. - It does **not typically present with joint pain or fever**, differentiating it from the presented symptoms.

Q: A 60-year-old female presenting with proximal muscle weakness, increased serum creatine kinase levels, and no skin manifestations is likely to have:

Polymyositis. ***Polymyositis*** - **Polymyositis** primarily affects adults, causing **proximal muscle weakness** (shoulders, hips, thighs) and elevated **creatine kinase (CK)** levels due to muscle damage [1]. - The absence of **skin manifestations** in this case strongly supports polymyositis over dermatomyositis, as dermatomyositis typically presents with characteristic cutaneous features [1]. *Dermatomyositis* - While dermatomyositis can present with identical **proximal muscle weakness** and elevated CK levels, it is characteristically associated with **skin manifestations** [1]. - The absence of **heliotrope rash**, **Gottron papules**, or **shawl sign** in this clinical scenario makes dermatomyositis less likely despite similar muscle involvement [1]. *Limb-girdle muscular dystrophy* - **Limb-girdle muscular dystrophy** is a genetically heterogeneous group of disorders, typically presenting in **childhood or adolescence**, though some forms can manifest later in life. - It causes **progressive symmetric muscle weakness** predominantly affecting the **shoulders and hips**, with highly variable CK levels depending on the specific genetic mutation. *Inclusion body myositis* - **Inclusion body myositis** typically affects older individuals (over 50), presenting with **progressive, asymmetrical muscle weakness**, more prominently in the **distal muscles** (e.g., forearm flexors, quadriceps). - While CK levels can be elevated, they are usually **less markedly increased** than in polymyositis or dermatomyositis, and the disease often progresses more slowly.

Q: Which of the following is a definite indication for systemic steroids in rheumatoid arthritis?

b) Lung involvement. ***Lung involvement*** - **Systemic steroids** are indicated for severe **extrapulmonary manifestations** of rheumatoid arthritis, such as **interstitial lung disease**, which can be life-threatening [2]. - They help to reduce **inflammation** and prevent further damage to lung tissue [1]. *Mononeuritis multiplex* - While a serious complication of rheumatoid arthritis, **mononeuritis multiplex** typically warrants treatment with disease-modifying antirheumatic drugs (DMARDs) and sometimes **intravenous immunoglobulins** (IVIG) or **cyclophosphamide**, rather than systemic steroids as a primary or definite indication. - Steroids might be used as an adjunct, but are not the definite first-line sole treatment. *Carpal tunnel syndrome* - **Carpal tunnel syndrome** in rheumatoid arthritis is often managed with **local corticosteroid injections**, **splinting**, or surgical release, depending on severity. - **Systemic steroids** are generally not indicated for localized musculoskeletal complications unless there is overwhelming active systemic disease. *Both 'a' & 'c'* - This option is incorrect because neither **mononeuritis multiplex** nor **carpal tunnel syndrome** is a definite standalone indication for systemic steroids in rheumatoid arthritis, as explained above. - Systemic steroids are reserved for more severe or diffuse manifestations.

Q: Behçet's disease is associated with which HLA?

HLA-B51. ***HLA-B51*** - Behçet's disease has a strong association with the **HLA-B51 allele**, particularly in populations from the Mediterranean, Middle East, and Asia. - This genetic predisposition contributes to the inflammatory and autoimmune nature of the disease. *HLA-B27* - This allele is strongly associated with **spondyloarthropathies**, such as **ankylosing spondylitis** and **reactive arthritis**, rather than Behçet's disease [1]. - Patients with HLA-B27 positive status often present with axial skeletal involvement and enthesitis [1]. *HLA-DQ* - HLA-DQ alleles are associated with various autoimmune conditions, including **celiac disease** and **type 1 diabetes mellitus**, but not specifically with Behçet's disease. - The association is generally weaker and less specific compared to HLA-B51 for Behçet's. *HLA-DR4* - **HLA-DR4** is primarily associated with **rheumatoid arthritis**, especially in severe forms of the disease. - It is also associated with other autoimmune diseases but does not play a significant role in the pathogenesis or diagnosis of Behçet's disease.

Q: What is the primary condition associated with atrophic changes in the mucous membranes?

Sjogren's syndrome. ***Sjogren's syndrome*** - It is an **autoimmune disease** primarily characterized by the destruction of exocrine glands, leading to **dryness of mucous membranes** (xerostomia, xerophthalmia) [1]. - The chronic inflammation and lymphocytic infiltration of glands result in **atrophy and dysfunction**, particularly of the salivary and lacrimal glands. *Lupus erythematosus* - While it is an autoimmune disease that can affect multiple organs, its primary manifestation is not typically **atrophic changes in mucous membranes**. [1] - **Oral ulcers** and **photosensitivity** are common, but widespread glandular atrophy is not characteristic [2]. *Celiac disease* - This is an **autoimmune disorder** primarily affecting the **small intestine** due to gluten ingestion, leading to **villous atrophy**. - While it can cause some oral manifestations (e.g., aphthous ulcers), it does not directly cause widespread atrophy of mucous membranes in other parts of the body. *Vitamin B12 deficiency* - Can cause **glossitis** (inflammation of the tongue), which might appear smooth and atrophic due to the loss of papillae. - However, it does not typically lead to systemic atrophy of mucous membranes beyond specific areas like the tongue or a general dryness.

Question 1

A 25-year-old female develops serous otitis media of the left ear, accompanied by cough, occasional hemoptysis, hematuria, and epistaxis, over a period of one and a half months. Her hemoglobin is 7 g, blood pressure is >170/100 mmHg, proteinuria is ++++, and she is negative for rheumatoid factor and antineutrophil cytoplasmic antibody. What is the likely cause?

Accepted Answer

Goodpasture's syndrome

Answer

Rapidly proliferative glomerulonephritis

Answer

Rheumatoid arthritis

Answer

Wegener's granulomatosis

Question 2

Anti-Ro antibodies are present in all except:

Accepted Answer

Mixed connective tissue disorder

Answer

SLE

Answer

Sjögren syndrome

Answer

Neonatal lupus

Question 3

A 20-year-old girl presents with abdominal pain and a purpuric rash all over the body, and the most probable diagnosis is?

Accepted Answer

Henoch-Schönlein Purpura (HSP)

Answer

Hemolytic Uremic Syndrome (HUS)

Answer

Kawasaki Syndrome

Answer

Immune Thrombocytopenic Purpura (ITP)

Question 4

All are true regarding lupus anticoagulant except?

Accepted Answer

Thrombocytosis

Answer

Arterial thrombosis

Answer

Increased antibodies

Answer

Rashes

Question 5

Lady presents with joint pain in both knees and low-grade fever, which occurs intermittently. On examination, she has a rash on sun-exposed areas. What is the clinical diagnosis?

Accepted Answer

Systemic lupus erythematosus (SLE)

Answer

Rheumatoid arthritis (RA)

Answer

Photodermatitis

Answer

Porphyria cutanea tarda

Question 6

A 60-year-old female presenting with proximal muscle weakness, increased serum creatine kinase levels, and no skin manifestations is likely to have:

Accepted Answer

Polymyositis

Answer

Dermatomyositis

Answer

Limb-girdle muscular dystrophy

Answer

Inclusion body myositis

Question 7

Which of the following is a definite indication for systemic steroids in rheumatoid arthritis?

Accepted Answer

b) Lung involvement

Answer

a) Mononeuritis multiplex

Answer

c) Carpal tunnel syndrome

Answer

d) Both 'a' & 'c'

Question 8

Behçet's disease is associated with which HLA?

Accepted Answer

HLA-B51

Answer

HLA-B27

Answer

HLA-DQ

Answer

HLA-DR4

Question 9

Which of the following statements is true regarding the use of combination therapy with anakinra and infliximab in patients with rheumatoid arthritis?

Accepted Answer

Combination therapy is not recommended due to the risk of serious infections.

Answer

Combination therapy is more effective than methotrexate plus etanercept.

Answer

Combination therapy is more effective than methotrexate alone.

Answer

Combination therapy has similar effectiveness to oral triple therapy.

Question 10

What is the primary condition associated with atrophic changes in the mucous membranes?

Accepted Answer

Sjogren's syndrome

Answer

Lupus erythematosus

Answer

Celiac disease

Answer

Vitamin B12 deficiency

Rheumatology and Immunology — MCQs

Rheumatology and Immunology — MCQs

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