Rheumatology and Immunology Practice Questions

Q: What is the best initial treatment for a patient with Behcet syndrome and ocular involvement?

Systemic glucocorticoids and azathioprine. Behcet syndrome is a multisystem inflammatory vasculitis characterized by recurrent oral and genital ulcers, skin lesions, and uveitis. The management strategy is dictated by the severity of organ involvement. **Why Systemic Glucocorticoids and Azathioprine are correct:** Ocular involvement (typically posterior uveitis or panuveitis) is considered a "major organ" manifestation that carries a high risk of permanent vision loss. Therefore, aggressive systemic therapy is required. **Systemic glucocorticoids** provide rapid control of acute inflammation, while **Azathioprine** is the preferred first-line steroid-sparing immunosuppressant to prevent relapses and long-term ocular damage. In severe or refractory cases, anti-TNF agents (Infliximab) or Cyclosporine may be used. **Why other options are incorrect:** * **Option A:** Topical steroids are sufficient for isolated minor oral ulcers or anterior uveitis, but they cannot penetrate the posterior segment of the eye or prevent systemic progression. * **Option B:** Thalidomide is effective for refractory mucocutaneous ulcers but is not the standard of care for ocular disease due to its side effect profile and lack of efficacy in preventing blindness. * **Option D:** Interferon-alfa is a second-line or adjunctive therapy for resistant cases; it is not the "best initial" treatment. **High-Yield Clinical Pearls for NEET-PG:** * **Pathergy Test:** A highly specific (but less sensitive) diagnostic test where a sterile needle prick results in a papule/pustule within 24–48 hours. * **HLA Association:** Strongly associated with **HLA-B51**. * **Classic Triad:** Recurrent oral ulcers, genital ulcers, and uveitis. * **Vascular involvement:** Behcet is unique as it involves both arteries and veins (e.g., Budd-Chiari syndrome, pulmonary artery aneurysms).

Q: What is the most common lymphoma associated with Sicca syndrome?

Mucosa-associated lymphoid tissue lymphoma (MALToma). Sicca syndrome (primary Sjögren’s syndrome) is characterized by chronic lymphocytic infiltration of the exocrine glands. Patients with Sjögren’s syndrome have a **44-fold increased risk** of developing non-Hodgkin lymphoma compared to the general population. **1. Why MALToma is correct:** The chronic antigenic stimulation of B-cells within the salivary glands leads to the formation of ectopic germinal centers. Over time, this persistent activation can result in a monoclonal B-cell proliferation, most commonly leading to **Extranodal Marginal Zone B-cell Lymphoma**, also known as **MALToma** [1]. These typically arise within the parotid glands. **2. Analysis of Incorrect Options:** * **Diffuse large B-cell lymphoma (DLBCL):** While Sjögren’s patients can develop DLBCL, it is usually a result of a high-grade transformation from a pre-existing MALToma. It is not the *most common* initial presentation. * **Burkitt lymphoma:** This is an aggressive B-cell lymphoma associated with EBV and c-myc translocation; it is not specifically linked to the chronic inflammatory environment of Sicca syndrome [1]. * **Lymphoplasmacytic lymphoma:** This is associated with Waldenström macroglobulinemia. While Sjögren’s involves plasma cell activity, this specific lymphoma is not the characteristic association. **High-Yield Clinical Pearls for NEET-PG:** * **Predictors of Lymphoma in Sjögren’s:** Persistent parotid gland enlargement, purpura, leukopenia, cryoglobulinemia, and low C4 complement levels. * **Most common site:** The parotid gland is the most frequent site for lymphoma development in these patients. * **Biomarker:** A decrease in previously high rheumatoid factor (RF) titers or the disappearance of hypergammaglobulinemia can paradoxically signal the onset of lymphoma.

Q: An 18-year-old boy presents with digital gangrene in the third and fourth fingers for the last 2 weeks. On examination, the blood pressure is 170/110 mm of Hg, and all peripheral pulses were palpable. Food and urine examinations were unremarkable. Antinuclear antibodies, antibody to double-stranded DNA, and antineutrophil cytoplasmic antibody were negative. What is the most likely diagnosis?

Polyarteritis nodosa. The clinical presentation of **Polyarteritis Nodosa (PAN)** is characterized by a medium-vessel vasculitis that typically spares the lungs and the glomeruli [1]. **Why Polyarteritis Nodosa (PAN) is correct:** 1. **Digital Gangrene:** PAN involves medium-sized arteries, leading to ischemia and infarction of distal extremities [2]. 2. **Hypertension:** This is a hallmark of PAN, resulting from renal artery involvement (renin-mediated) rather than glomerulonephritis. 3. **Palpable Pulses:** Unlike large-vessel vasculitis, peripheral pulses are usually preserved because the primary pathology is in smaller branch arteries. 4. **Negative Serology:** PAN is characteristically **ANCA-negative** (unlike Wegener’s) and **ANA/dsDNA-negative** (unlike SLE). The "unremarkable urine" confirms the absence of glomerulonephritis, a key diagnostic feature that distinguishes PAN from microscopic polyangiitis. **Why other options are incorrect:** * **Wegener’s Granulomatosis (GPA):** Typically presents with upper/lower respiratory tract involvement and is strongly associated with **c-ANCA (PR3)**. * **Takayasu’s Arteritis:** A large-vessel vasculitis that usually presents with **absent/diminished pulses** ("pulseless disease") and is more common in young females. * **Systemic Lupus Erythematosus (SLE):** While it can cause vasculitis, the negative ANA and dsDNA, along with the absence of proteinuria/hematuria, make it highly unlikely. **High-Yield Clinical Pearls for NEET-PG:** * **PAN Association:** Strongly associated with **Hepatitis B** (HBV) surface antigenemia (10–30% of cases). * **Angiography:** Shows characteristic **"string of pearls"** appearance due to multiple microaneurysms. * **Sparing:** PAN classically **spares the lungs** (no pulmonary involvement). * **Biopsy:** Gold standard for diagnosis, showing transmural necrotizing inflammation.

Q: Which of the following findings is NOT seen in Dermatomyositis?

Salmon rash. **Explanation:** The correct answer is **Salmon rash**, as this is a characteristic clinical finding of **Adult-Onset Still’s Disease (AOSD)**, not Dermatomyositis. The salmon-colored, maculopapular, non-pruritic rash typically appears during febrile spikes on the trunk and limbs. **Why the other options are incorrect (Findings in Dermatomyositis):** * **Gottron’s papules (Option A):** These are pathognomonic for Dermatomyositis [1]. They are erythematous to violaceous scaly papules found over the dorsal aspects of the MCP, PIP, and DIP joints [2]. * **Mechanic’s hands (Option B):** Characterized by hyperkeratotic, fissured, and scaling skin on the lateral and palmar aspects of the fingers. While seen in Dermatomyositis, it is highly associated with **Anti-synthetase syndrome** (Anti-Jo-1 antibodies) [1]. * **Periungual telangiectasia (Option C):** These are dilated capillary loops at the nail folds, often accompanied by cuticular hypertrophy [2]. They reflect the underlying systemic microvascular involvement of the disease. **High-Yield Clinical Pearls for NEET-PG:** 1. **Heliotrope Rash:** A violaceous eruption on the upper eyelids, often with periorbital edema; another pathognomonic sign [2]. 2. **Shawl Sign & V-sign:** Photosensitive erythematous rashes over the posterior neck/shoulders and the anterior chest, respectively [2]. 3. **Malignancy Risk:** Dermatomyositis has a strong association with internal malignancies (e.g., ovarian, lung, breast, GI); age-appropriate cancer screening is mandatory [2]. 4. **Antibody Profile:** **Anti-Mi-2** is highly specific for Dermatomyositis and carries a good prognosis.

Q: Leukopenia in Systemic Lupus Erythematosus (SLE) is almost always associated with which specific cell type deficiency?

Lymphopenia. In Systemic Lupus Erythematosus (SLE), hematological abnormalities are a hallmark of the disease and are included in the ACR/SLICC classification criteria. **Lymphopenia** (defined as <1,500/mm³) is the most characteristic and frequent cause of leukopenia in SLE [1]. **1. Why Lymphopenia is the correct answer:** Lymphopenia in SLE is primarily mediated by **anti-lymphocyte antibodies** (Type II hypersensitivity). These autoantibodies target surface antigens on T and B cells, leading to their destruction or sequestration. It is a highly specific marker of disease activity; a falling lymphocyte count often correlates with an impending SLE flare [1]. **2. Why other options are incorrect:** * **Neutropenia:** While it can occur in SLE, it is much less common than lymphopenia. When present, it is often mild or related to medications (e.g., immunosuppressants) rather than the primary disease process itself. * **Eosinopenia:** This is not a characteristic feature of SLE. Eosinopenia is more commonly associated with acute stress, Cushing’s syndrome, or the use of glucocorticoids. * **Any of the above:** While multiple cell lines can be affected (pancytopenia), lymphopenia is the "almost always" associated finding that defines the leukopenic state in the context of SLE diagnostic criteria [1]. **Clinical Pearls for NEET-PG:** * **Hematological Criteria for SLE:** Leukopenia (<4,000/mm³), Lymphopenia (<1,500/mm³), Thrombocytopenia (<100,000/mm³), and Autoimmune Hemolytic Anemia [1]. * **Anemia of Chronic Disease** is the most common cause of anemia in SLE, but **Autoimmune Hemolytic Anemia (Coombs positive)** is the one included in the diagnostic criteria. * **High-Yield Fact:** If a patient with SLE presents with sudden, severe neutropenia, always rule out **Felty’s Syndrome** (though more common in RA) or drug-induced marrow suppression.

Q: Which of the following is an ANCA negative vasculitis?

Polyarteritis nodosa. The classification of systemic vasculitis is primarily based on the size of the vessels involved and the presence of specific biomarkers like **Anti-Neutrophil Cytoplasmic Antibodies (ANCA)** [2]. **1. Why Polyarteritis Nodosa (PAN) is the correct answer:** Polyarteritis nodosa is a **medium-vessel vasculitis**. By definition, it is **ANCA-negative**. It is characterized by necrotizing inflammation of medium-sized or small arteries without glomerulonephritis or vasculitis in arterioles, capillaries, or venules. A key diagnostic feature is its association with **Hepatitis B virus (HBV)** infection and the presence of "rosary sign" (microaneurysms) on angiography. **2. Why the other options are incorrect:** Options A, B, and D belong to the category of **ANCA-Associated Vasculitides (AAV)**, which primarily affect small vessels: * **Wegener Granulomatosis (Granulomatosis with Polyangiitis - GPA):** Strongly associated with **c-ANCA** (anti-PR3). It typically involves the triad of upper respiratory tract, lungs, and kidneys. * **Churg-Strauss Syndrome (Eosinophilic Granulomatosis with Polyangiitis - EGPA):** Associated with **p-ANCA** (anti-MPO) [1]. It is characterized by asthma, peripheral eosinophilia, and granulomatous inflammation [1]. * **Microscopic Polyangiitis (MPA):** Strongly associated with **p-ANCA** (anti-MPO). Unlike PAN, it involves small vessels (capillaries) and frequently causes pauci-immune necrotizing glomerulonephritis. **High-Yield Clinical Pearls for NEET-PG:** * **PAN Rule of Thumb:** "PAN is ANCA-negative and spares the lungs." * **c-ANCA (Cytoplasmic):** Targets Proteinase-3 (PR3); specific for GPA. * **p-ANCA (Perinuclear):** Targets Myeloperoxidase (MPO); seen in MPA, EGPA, and Primary Sclerosing Cholangitis [1]. * **Microaneurysms:** In PAN, these are commonly found in renal, mesenteric, or hepatic arteries.

Q: Which of the following is NOT an indication for intravenous immunoglobulin (IVIg)?

Systemic lupus erythematosus (SLE). **Explanation:** The correct answer is **Systemic lupus erythematosus (SLE)**. While IVIg is used in various autoimmune and inflammatory conditions, it is **not** considered a standard or first-line indication for SLE [1]. SLE is primarily managed with corticosteroids, hydroxychloroquine, and immunosuppressants (like mycophenolate mofetil or cyclophosphamide) or biologics (like Belimumab). **Why the other options are incorrect (Indications for IVIg):** * **Idiopathic Thrombocytopenic Purpura (ITP):** IVIg is a first-line treatment, especially when a rapid increase in platelet count is required (e.g., pre-surgery or life-threatening bleed). It works by blocking Fc receptors on splenic macrophages, preventing platelet destruction. * **Kawasaki Disease:** High-dose IVIg administered within the first 10 days of fever onset is the gold standard treatment. It significantly reduces the risk of developing **coronary artery aneurysms**. * **Acute Infective Polyneuritis (Guillain-Barré Syndrome):** IVIg is a mainstay of treatment, equivalent in efficacy to plasmapheresis. It helps neutralize pathogenic autoantibodies and modulate complement activation. **NEET-PG High-Yield Pearls:** 1. **Mechanism of Action:** IVIg provides passive immunity, neutralizes circulating autoantibodies, and inhibits complement-mediated damage. 2. **Other Key Indications:** Dermatomyositis (refractory), Myasthenia Gravis (crisis), Chronic Inflammatory Demyelinating Polyneuritis (CIDP), and Common Variable Immunodeficiency (CVID). 3. **Side Effect:** A classic board-favorite side effect of IVIg is **Aseptic Meningitis**. 4. **Contraindication:** Patients with **IgA deficiency** are at risk of anaphylaxis when given IVIg due to the presence of anti-IgA antibodies.

Question 1

What is the best initial treatment for a patient with Behcet syndrome and ocular involvement?

Accepted Answer

Systemic glucocorticoids and azathioprine

Answer

Topical glucocorticoids to the oral ulcers and conjunctiva

Answer

Thalidomide

Answer

Intralesional interferon alfa

Question 2

What is the most common lymphoma associated with Sicca syndrome?

Accepted Answer

Mucosa-associated lymphoid tissue lymphoma (MALToma)

Answer

Diffuse large B-cell lymphoma (DLBCL)

Answer

Burkitt lymphoma

Answer

Lymphoplasmacytic lymphoma

Question 3

Which polyarthritic condition is not common?

Accepted Answer

Systemic lupus erythematosus (SLE)

Answer

Gout

Answer

Psoriatic arthritis

Answer

Ankylosing spondylitis

Question 4

A 28-year-old woman presents with a recent episode of coughing up blood, frequent nosebleeds, and decreased urine output. Physical examination reveals an ulcerated nasal mucosa. Urinalysis is positive for protein and red blood cells, consistent with acute glomerulonephritis. Chest X-ray shows two cavitary lesions, and serology is positive for antineutrophil cytoplasmic antibodies (ANCA). Which of the following is the most likely diagnosis?

Accepted Answer

Wegener granulomatosis

Answer

Bacterial endocarditis

Answer

Goodpasture syndrome

Answer

Lupus erythematosus

Question 5

An 18-year-old boy presents with digital gangrene in the third and fourth fingers for the last 2 weeks. On examination, the blood pressure is 170/110 mm of Hg, and all peripheral pulses were palpable. Food and urine examinations were unremarkable. Antinuclear antibodies, antibody to double-stranded DNA, and antineutrophil cytoplasmic antibody were negative. What is the most likely diagnosis?

Accepted Answer

Polyarteritis nodosa

Answer

Wegener's granulomatosis

Answer

Takayasu's arteritis

Answer

Systemic lupus erythematosus (SLE)

Question 6

Which of the following findings is NOT seen in Dermatomyositis?

Accepted Answer

Salmon rash

Answer

Gottron's papules

Answer

Mechanic's hands

Answer

Periungual telangiectasia

Question 7

Leukopenia in Systemic Lupus Erythematosus (SLE) is almost always associated with which specific cell type deficiency?

Accepted Answer

Lymphopenia

Answer

Neutropenia

Answer

Eosinopenia

Answer

Any of the above

Question 8

Which of the following is an ANCA negative vasculitis?

Accepted Answer

Polyarteritis nodosa

Answer

Wegener granulomatosis

Answer

Churg-Strauss syndrome

Answer

Microscopic polyangiitis

Question 9

A 40-year-old male complains of exquisite pain and tenderness over the left ankle. There is no history of trauma. The patient is taking a mild diuretic for hypertension. On examination, the ankle is very swollen and tender. There are no other physical examination abnormalities. What is the next step in management?

Accepted Answer

Obtain uric acid level and perform arthrocentesis

Answer

Begin colchicine and broad-spectrum antibiotics

Answer

Begin allopurinol if uric acid level is elevated

Answer

Obtain ankle x-ray to rule out fracture

Question 10

Which of the following is NOT an indication for intravenous immunoglobulin (IVIg)?

Accepted Answer

Systemic lupus erythematosus (SLE)

Answer

Idiopathic thrombocytopenic purpura (ITP)

Answer

Kawasaki disease

Answer

Acute infective polyneuritis

Rheumatology and Immunology — MCQs

Rheumatology and Immunology — MCQs

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